ABIM 2015 - Heme/Onc

Question 1

What does the surface marker CD34 say about that cell?

Answer 1

That it is a STEM cell

Question 2

Aplastic Anemia, Pure Red Cell Aplasia, Neutropenia and Thrombocytopenia are all caused by what failure?

Answer 2

Bone Marrow Failure

Question 3

PANcytopenia with hypocellular bone marrow caused by Heredity, Idiopathic, Immune Dysfunction (Drugs, Infections, Toxins or Radiation)?

Answer 3

Aplastic Anemia

Question 4

The hematopoietic, pluripotent STEM cell gives rise to two STEM cells, the MYELOID STEM Cell and the LYMPHOID STEM Cell. What does the LYMPHOID STEM Cell give rise to?

Answer 4

B & T Lymphocytes ONLY

Question 5

RBCs (Erythrocytes), WBCs (Granulocytes, Monocytes, Basophils, Eosinophils) and PLATELETS come from what STEM Cells?

Answer 5

MYELOID STEM Cells

Question 6

What is another name of GRANULOCYTE (part of the WBCs and therefore the Myeloid lineage)?

Answer 6

NEUTROPHIL (Segments and Bands or “Stabs”)

Question 7

Neutrophils (granulocytes) + Basophils + Eosinophils = ?

Answer 7

Polymorphonuclear cells (PMNs) - their Nuclei are MULTI-lobulated

Question 8

What do FEMALE Neutrophils (granulocytes) have that MALE Neutrophils DO NOT?

Answer 8

X-chromosome structure

Question 9

What is the NORMAL number of segments that a mature Neutrophil has?

Answer 9

3-5

Question 10

What is an IMMATURE Neutrophil called?

Answer 10

A “band” or “stab” cell

Question 11

What is the most ABUNDANT WBC in the blood?

Answer 11

The Neutrophil

Question 12

What is a reticulocyte?

Answer 12

An immature RBC (that has a nucleus - mature RBCs do not)

Question 13

What is the Absolute Neutrophil Count (ANC) of Moderate Aplastic Anemia?

Answer 13

500-1,000/µL

Question 14

What is the Absolute Neutrophil Count (ANC) of Severe Aplastic Anemia?

Answer 14

200-500/µL

Question 15

What is the Absolute Neutrophil Count (ANC) of VERY Severe Aplastic Anemia?

Question 16

The most common CONGENITAL Aplastic Anemia with Skin defects, Short stature, HYPOgonadism, MICROcephaly and Urogenital abnormalities is called?

Answer 16

FANCONI Anemia

Question 17

Fatigue, Exertional Dyspnea, Bleeding and Infections are seen in this anemia?

Answer 17

Aplastic Anemia

Question 18

What MUST a CBC demonstrate to diagnose Aplastic Anemia?

Answer 18

Neutropenia AND Thrombocytopenia (or, PANcytopenia)

Question 19

How is Aplastic Anemia diagnosed?

Answer 19

Bone Marrow aspirate AND Biopsy

Question 20

What do patients with classic Paroxysmal Nocturnal Hemoglobinuria (PNH) develop later?

Answer 20

Aplastic Anemia

Question 21

When is treatment for Paroxysmal Nocturnal Hemoglobinuria (PNH) started?

Answer 21

When HEMOLYSIS occurs

Question 22

How should patients YOUGER than 40 with an HLA-compatible sibling be treated for Severe Aplastic Anemia?

Answer 22

By Allogenic (same species) Hematopoietc Stem Cell Transplantation (75-90% CURE)

Question 23

How should patients who are NOT candidates for Stem Cell Transplantation be treated for Severe Aplastic Anemia?

Answer 23

Immunosuppressants (anti-Thymocyte globulin and Cyclosporine) antithymocyte globulin is animal-derived Ab against human T-cells

Question 24

Pts who survive Aplastic Anemia have a risk of developing these two diseases?

Answer 24

Myelodysplasia and AML

Question 25

What are the prophylactic agents used in patients with Aplastic Anemia?

Answer 25

Antibiotic, Antiviral and Anti-fungal agents

Question 26

What type of transfusions MUST be given to pts with Aplastic Anemia if they require blood transfusion?

Answer 26

IRRADIATED, Leukocyte DEPLETED transfusions (of RBCs and Platelets)

Question 27

Below what platelet number should pts with Aplastic Anemia receive PLATELET transfusions?

Answer 27

HIGH-Risk or if Platelets

Question 28

Bone Marrow Aspirate and Biopsy shows a HYPOcellular bone marrow with Increased FAT space and Decreased hematopoietic elements?

Answer 28

Aplastic Anemia

Question 29

Severe NORMOCYTIC anemia with lack of reticulocytes and no eryhtroid precursors in the bone marrow without any effect on leukocyte or platelets?

Answer 29

Pure RED Cell Aplasia

Question 30

Medications, Toxins, Thymoma, Parvovirus B19, HIV and Autoimmune disorders are all associated with this ANEMIA type?

Answer 30

Pure RED Cell Aplasia

Question 31

What diagnostic tests should be done to confirm Pure RED Cell Aplasia?

Answer 31

CT (to exclude thymoma), Peripheral Blood of Bone Marrow Flow Cytometry and serologic evaluation for autoimmune disorders

Question 32

What should you test a patient for who already has an underlying Hemolytic Anemia and presents with an ACUTE severe HYPOproliferative anemia (deficient erythropoietin)?

Answer 32

Parvovirus B19

Question 33

What type of Anemias are caused by Erythropoietin deficiency?

Answer 33

NORMOCYTIC

Question 34

A patient with Pure RED Cell Aplasia was found to have a large number of granular LYMPHOCYTES with abundant cytoplasm and azurophilic granules infiltrating the bone marrow and also found on a peripheral smear?

Answer 34

Large Granular Lymphocytosis (common, malignant cause of Pure RED Cell Aplasia) - CD57-positive T-cells are diagnostic

Question 35

How is Pure RED Cell Aplasia treated?

Answer 35

Treat the underlying cause AND TRANSFUSE RBCs as well as Immunosuppressants (Cyclophosphamide or Cyclosporine or PREDNISONE or antithymocyte globulin)

Question 36

How is Parvovirus B19 treated in the normal patient and in immunocompromised patient?

Answer 36

No treatment necessary if immunocompetent, otherwise IVIG

Question 37

What medications can CAUSE Pure RED Cell Aplasia?

Answer 37

Exogenous Erythropoietin (Anti-EPO Ab form), phenytoin, Isoniazid, chloramphenicol

Question 38

What is the danger for a patient that is NEUTROPENIC?

Answer 38

Neutrophils are the MAJORITY of WBC's so BACTERIAL and FUNGAL infections

Question 39

Below what Number of Absolute Neutrophils (normal is 2,500-6,000/µL) is a patient at great risk of infections?

Question 40

In some Blacks, Yemenite Jews and Arabs the Absolute Neutrophil Count (ANC) is genetically low (1,000-1,500/µL) what should be done?

Answer 40

NOTHING AT ALL (only

Question 41

SLE and meds (CHLORAMPHENICOL, TMP-SMX, Cephalosporins, Chemotherapy drugs, Naproxen, propylthiouracil, carbamazepine, phenytoin, amiodarone and procainamide) can ALL cause what type of SERIOUS ANEMIA?

Answer 41

NEUTROPENIA (severely decreased Absolute Neutrophil Count - with high risk for infection)

Question 42

RA with Splenomegaly AND Neutropenia?

Answer 42

FELTY Syndrome

Question 43

How is Neutropenia diagnosed?

Answer 43

Bone Marrow Evaluation

Question 44

How is Neutropenia treated?

Answer 44

STOP offending drug or treat Underlying disease

Question 45

Although treating patients with SEVERE Congenital Neutropenia (ANC

Answer 45

Myelodysplasia and AML

Question 46

Is ISOLATED Thrombocytopenia associated with a Bone Marrow or Stem Cell disorder?

Answer 46

NO!! (these are usually acquired - meds, toxins, etc.)

Question 47

Chemotherapy, radiation, inherited defects in DNA repair (FANCONI Anemia), and Down Syndrome can all have this Hematopoietic STEM Cell disorder?

Answer 47

Myelodysplastic Syndrome

Question 48

What does Myelodysplastic Syndrome (Myelodysplasia) transform to?

Answer 48

AML (associated with Sweet Syndrome - neutrophillic infiltrate on biopsy)

Question 49

Bone Marrow biopsy revealing HYPERcellularity and Dysplasia (abnormal cells) of Myeloid lineage (erythrocytes, neutrophils, basophils, eosinophils, platelets) is what?

Answer 49

Myelodysplasia

Question 50

The NUMBER of affected cell lineages (neutrophils, platelets, erythrocytes, basophils, eosinophils), amount of Blasts (immature cells) and advanced age are associated with what?

Answer 50

Prognosis of a patient with Myelodysplasia (if >5% blasts or multilineages - poor prognosis)

Question 51

When should IRON chelation therapy be started?

Answer 51

When serum ferritin level ≥1,000 ng/mL

Question 52

How are the anemias of Myelodysplasia treated?

Answer 52

RBC or Platelet transfusions (can cause iron overload)

Question 53

What is considered a "Dysplastic" neutrophil?

Answer 53

One that has a decreased amount of granules and 5 segmented lobes of the nucleus

Question 54

What is a HYPOgranular Neutrophil with a bi-lobed nucleus in a patient with Myelodysplasia called?

Answer 54

Pelger-Huet morphology

Question 55

What two chemotherapeutic agents are BEST for treating Myelodysplasia?

Answer 55

AZAcitidine and DECitabine (decreased progression to AML and less transfusions required)

Question 56

What is the BEST treatment for the LOWER-Risk, transfusion-dependent Myelodysplasia with Chromosome 5q deletion?

Answer 56

LENALinomide

Question 57

What GENETIC feature predicts good response to Immunosuppressive therapy in patients with Myelodysplasia?

Answer 57

HLA-DR15 and younger age

Question 58

What is CURATIVE treatment in patients with Myelodysplasia but is reserved for YOUNGER patients with HIGH-RISK disease due to its high mortality risk?

Answer 58

Allogenic (same species) Hematopoietic Stem Cell Transplantation (HSCT)

Question 59

A patient that presents with features of Myelodysplasia BUT has Weight LOSS, Sweats and Splenomegaly with LEUKOCYTOSIS and HIGH Monocyte count likely has?

Answer 59

Chronic MyeloMonocytic Leukemia (CMML)

Question 60

What hematologic condition is associated with Down Syndrome that eventually transforms to AML?

Answer 60

Myelodysplasia

Question 61

What should be done for a patient with Myelodysplasia with LOW-Risk disease who is asymptomatic?

Answer 61

OBSERVATION ONLY

Question 62

What agents can improve Hb in patients with lower-risk Myelodysplasia with SYMPTOMATIC Anemia?

Answer 62

Erythropoiesis-stimulants (Epoetin and Darbepoetin)

Question 63

A Myeloproliferative disorder in which ALL of the circulating erythrocytes, leukocytes and platelets (all the myeloid lineages) are derived from a SINGLE NEOPLASTIC STEM CELL and can grow and divide WITHOUT erythropoetin?

Answer 63

Polycythemia Vera

Question 64

Hyperviscosity of blood with thrombosis in a patient 50-75 years old with bone marrow fibrosis and HEPATOSPLENOMEGALY (because hematopoietic activity is driven into the liver and spleen) with PRURITUS after BATHING, BURNING of the PALMS and SOLES (erythromelalgia) with FEVER, WEIGHT LOSS and SWEATING, GI BLEEDING, confusion, TIA-like symptoms, tinnitus, blurred vision and HA?

Answer 64

Polycythemia Vera

Question 65

What liver disorder can Polycythemia Vera cause BESIDES Hepatomegaly?

Answer 65

Budd-Chiari Syndrome (hepatic venous outflow obstruction)

Question 66

Elevated Hct with MICROCYTOSIS and a LOW or Undetectable Erythropoetin level with normal oxygen Saturation with elevated serum uric acid and Vit B12 levels?

Answer 66

Polycythemia Vera

Question 67

JAK2 protein MUTATION?

Answer 67

Polycythemia Vera

Question 68

Is Polycythemia Vera a possibility if the JAK2 protein mutation is absent and erythropoietin level is normal or high?

Answer 68

NO!!

Question 69

What are mimics of Polycythemia Vera which are called Secondary Erythrocytosis?

Answer 69

Sleep Apnea, Emphysema, CO-poisoning, Smoking, Renal Artery Stenosis

Question 70

What medication decreases thrombosis in Polycythemia Vera and should be given to ALL patients?

Answer 70

ASPIRIN

Question 71

What therapy provides the BEST overall survival for Polycythemia Vera?

Answer 71

Phlebotomy 1-2x/week until Hct

Question 72

What can be used to treat Polycythemia Vera in PREGNANCY?

Answer 72

Interferon-alpha

Question 73

What else besides Aspirin and Phlebotomy should be added in patients >60 years old who have had previous thrombotic episodes?

Answer 73

Hydroxyurea

Question 74

Elevated PLATELET count (≥600,000/µL) on TWO occasins 1 MONTH apart, with normal platelets due to INCREASED Megakaryocyte production with microvascular thrombi, DIGITAL Ischemia, hemorrhagic symptoms and splenomegaly?

Answer 74

Essential Thrombocythemia

Question 75

How is Essential Thrombocythemia treated?

Answer 75

Anti-platelet agents

Question 76

What condition can Iron deficiency, chronic bleeding, cancer, inflammation, infection or post-splenectomy mimic and must be excluded?

Answer 76

Essential Thrombocythemia

Question 77

The ABSENCE of what chromosome excludes CML in the setting of Essential Thrombocythemia?

Answer 77

Philadelphia Chromosome

Question 78

What disease does Polycythemia Vera (like Myelodysplasia) progress to?

Answer 78

AML

Question 79

Does Essential Thrombocythemia progress to AML (like Polycythemia Vera and Myelodysplasia do)?

Answer 79

NO!!

Question 80

What is the MAJOR complication of Essential Thrombocythemia?

Answer 80

Venous and arterial thrombosis

Question 81

When in patients with Essential Thrombocythemia do you see PSEUDOhyperkalemia and PSEUDOhypoglycemia and bizzare platelet morphology?

Answer 81

When platelet counts ≥1-1.5 MILLION (because platelets use glucose and release potassium)

Question 82

Clusters of ABNORMAL megakaryocytes in the bone marrow are found in what disease?

Answer 82

Essential Thrombocythemia

Question 83

What is the BEST therapy for Essential Thrombocythemia?

Answer 83

Hydroxyurea + Aspirin

Question 84

What therapy can be used in PREGNANT women who have Essential Thrombocythemia (also used for Polycythemia Vera?

Answer 84

Interferon-alpha

Question 85

What medication can be used for the treatment of Essential Thrombocythemia and Polycythemia Vera in patients who are older and CANNOT tolerate the side effects of other drugs?

Answer 85

32P (radioactive isotope) - HIGH risk of leukemia

Question 86

What leukemia is the Philadelphia Chromosome seen in?

Answer 86

CML t(9;22) BCR-ABL tyrosine kinase

Question 87

What two other Leukemias does CML manifest as during the BLAST phase?

Answer 87

AML (80%) and ALL (20%)

Question 88

SPLENOMEGALY, fatigue, night sweats, weight loss, abdominal discomfort, early satiety and bleeding with HIGH leukocyte count, HYPERcellular bone marrow with myeloid hyperplasia?

Answer 88

CML (chronic phase)

Question 89

How is CML diagnosed?

Answer 89

By demonstrating either t(9;22) or BCR-ABL transcript

Question 90

What patients, if diagnosed with CML, require treatment?

Answer 90

ALL of them, EVEN if ASYMPTOMATIC

Question 91

What are the BEST three drugs that treat CML?

Answer 91

BCR-ABL inhibitors (imaTINIB, niloTINIB, dasaTINIB)

Question 92

In patients who cannot tolerate the BCR-ABL inhibitors (-"tinib") or are in the accelerated or bast phases of CML rather than the chronic phase, what can be done?

Answer 92

Allogenic Hematopoietic Stem Cell Transplantation (HSCT)

Question 93

What is the most common PHYSICAL EXAMINATION finding in patients with CML?

Answer 93

Splenomegaly

Question 94

Abnormal MYELOID and MEGAKARYOCYTE proliferation stimulating collagen production, bone marrow fibrosis and therefore impaired bone marrow function driving hematopoiesis into the spleen and liver with resultant HEPATOSPLENOMEGALY and bone pain?

Answer 94

Myelofibrosis

Question 95

Anemia with nucleated erythrocytes and TEAR-DROP erythrocytes, elevated LDH, Uric acid and Alk Phos (bone)?

Answer 95

Myelofibrosis

Question 96

How is Myelofibrosis diagnosed?

Answer 96

Bone marrow biopsy, also has JAK2 mutation (as with Polycythemia Vera and Essential Thrombocythemia)

Question 97

How is Myelofibrosis treated?

Answer 97

Symptomatic (transfusions, hydroxyurea - for symptomatic hepatosplenomegaly and thrombocytosis, Danazol)

Question 98

This medication is used in myeloproliferative diseases, especially Polycythemia Vera and Essential Thrombocythemia as well as for symptomatic relief of the effects of hepatosplenomegaly and throbocytosis in those patients, also used in Sickle Cell anemia as it breaks down cells that sickle and increases production of fetal Hb?

Answer 98

Hydroxyurea

Question 99

What is a CURATIVE treatment for Myelofibrosis but as usual, has a high rate of morbidity and mortality in oder patients?

Answer 99

Allogenic Hematopoietic Stem Cell Transplantation (HCST)

Question 100

What is done for treatment of asymptomatic patients with Myelofibrosis?

Answer 100

Observation ONLY

Question 101

What disease is caused in part by the abnormal proliferation of megakaryocytes that causes excess fibroblast growth factors stimulating collagen production and therefore marrow fibrosis driving hematopoiesis into the liver and spleen?

Answer 101

Myelofibrosis

Question 102

What leukemia is associated with the presence of Auer Rods and circulating myeloblasts?

Answer 102

AML

Question 103

Having had Myelodysplasia (or other bone marrow failure syndromes) or a Myeloproliferative disorder (Polycythemia Vera, Essential Thrombocythemia), Fanconi Anemia (DNA repair defect) and Down Syndrome places one at risk for this disease?

Answer 103

AML

Question 104

Violaceous non-tender cutaneous plaques and gingival hyperplasia can be seen in a patient with what leukemia?

Answer 104

AML

Question 105

What neutrophilic dermatologic condition can be seen in AML or in Myelodysplasia that is about to convert to AML?

Answer 105

Sweet Syndrome

Question 106

Tumor lysis syndrome and DIC with severe cytopenias, hypercellular bone marrow showing a monotonous population of blasts (≥20%) or promyelocytes?

Answer 106

AML

Question 107

What cytochemical stain can differentiate myeloblasts from lymphoblasts?

Answer 107

Myeloperoxidase

Question 108

What patients have a favorable survival of AML?

Answer 108

YOUNGER than 55, t(8;21), t(15;17)-APL or inv(16)

Question 109

How are all leukemias treated supportively?

Answer 109

Transfusions when needed and management of deficient cells (infections in pts with neutropenias or anti-platelet agents in pts with thrombocythemias)

Question 110

What is TUMOR LYSIS SYNDROME and what conditions is it seen in?

Answer 110

Breakdown products of dying cells causing acute renal failure with ELEVATED (potassium, phos, uric acid) and LOW (calcium); caused by AML and the treatment of certain cancers (lymphomas, leukemias)

Question 111

How is Tumor Lysis Syndrome [(caused by the breakdown products of dying cells resulting in acute renal failure with ELEVATED (potassium, phos, uric acid) and LOW (calcium)] treated?

Answer 111

IVF, RASBURICASE, and ALLOPURINOL

Question 112

The leukocytosis that can be seen in CML and AML, especially when >50,000/µL can cause what symptoms?

Answer 112

CNS and Respiratory (HA, visual, AMS, hypoxia)

Question 113

How is DIC managed in patients with AML?

Answer 113

Transfusions (if Acute PROmyelocytic Leukemia, can add ALL-trans-retinoic acid)

Question 114

How are symptoms (CNS, pulmonary) of leukocytosis (>50,000/µL) in AML or CML treated?

Answer 114

Hydroxyurea or Leukapheresis

Question 115

How is AML treated?

Answer 115

7-days of cytarabine and 3-days of an anthracycline -"rubicin")

Question 116

What is "Consolidation" therapy in oncology?

Answer 116

Chemotherapy used to eradicate minimal RESIDUAL disease such as seen in AML after the ("7 and 3") treatment

Question 117

Why is CYTARABINE (standard induction chemotherapy used in AML) not used in adults >55?

Answer 117

Because of cerebellar toxicity and increased mortality

Question 118

What is considered CURATIVE treatment for AML?

Answer 118

Allogenic Hematopoietic Stem Cell Transplantation (HSCT)

Question 119

How is Acute ProMyelocytic Leukemia (APL)-a variant of AML-distinguished from Acute Myelocytic Leukemia (AML)?

Answer 119

By the presence of t(15;17) - more favorable outcome in patients

Question 120

The addition of WHAT drug to STANDARD induction chemotherapy (7-days cytarabine + 3-days of an anthracycline -"rubicin") can produce an 80% cure rate in patients with Acute ProMyelocytic Leukemia (APL)?

Answer 120

All-trans-retinoic acid (ALTRA) or arsenic trioxide (QT-prolongation)

Question 121

Blood or bone marrow with ≥20% myeloblasts is a diagnosis of?

Answer 121

AML

Question 122

Symptoms over days to weeks of fatigue, dyspnea, bleeding, infections and fever with sweats and weight loss are related to what?

Answer 122

Bone marrow failure

Question 123

Lymphadenopathy with Hepatosplenomegaly, large anterior mediastinal mass causing Superior Vena Cava syndrome (obstruction of the SVC causing facial edema, venous distention in the neck, UE edema, HA and dyspnea), pleural effusions with circulating LYMPHOblasts and tumor lysis syndrome?

Answer 123

ALL (acute LYMPHOblastic Leukemia)

Question 124

≥25% LYMPHOblasts on bone barrow examination is a diagnosis of what?

Answer 124

ALL (Acute Lymphoblastic Anemia)

Question 125

Why would ALL be NEGATIVE for myeloperoxidase test?

Answer 125

Because it is a LYMPHOblastic disease not MYELOblastic

Question 126

What are poor prognostic factors in a patient with ALL besides age >55?

Answer 126

A LEUKOCYTE count >30,000/µL and B-lymphocyte disease rather than T-lymphocyte disease

Question 127

What happens in BOTH AML and ALL shortly after the commencement of chemotherapy?

Answer 127

Tumor Lysis Syndrome (treat with IVF, Allopurinol and Rasburicase)

Question 128

What patients CANNOT use RASBURICASE (to treat tumor lysis syndrome) because it induces HEMOLYSIS?

Answer 128

Those with a glucose-6 phosphate dehydrogenase deficiency (G6PD)

Question 129

What feature can ALL (RARELY) have that is usually seen in CML patients and requires addition of additional chemotherapy agent use?

Answer 129

Philadelphia Chromosome t(9;22) BCR-ABL (-"tinib")

Question 130

Anthracycline (-"rubicin") + Vincristine + L-asparginase + Corticosteroid is the chemotherapeutic regimen for what leukemia?

Answer 130

ALL (add a -"tinib" if Philadelphia Chromosome positive)

Question 131

What CURATIVE therapy can be given to patients with high-risk ALL that are otherwise healthy and younger than 55?

Answer 131

Allogenic Hematopoietic Stem Cell Transplantation (HSCT)

Question 132

Because of the RISK of CNS involvement in patients with ALL, how is chemotherapy given ± radiation therapy?

Answer 132

Intrathecally (into spinal canal)

Question 133

What patients WITH CANCER should be given Erythropoiesis Stimulating Agents (epoetin or darbapoetin) and whom should these NOT be given to and why?

Answer 133

Only those patients WITH CANCER who suffer from CHEMOTHERAPY-related anemia (Hb ≤10) for whom the treatment is NOT curative (because it causes thromboembolism and poor tumor control)

Question 134

What patients WITHOUT CANCER are Erythropoiesis Stimulating Agents (epoetin and darbapoetin) used in?

Answer 134

Those with anemia (Hb≤10) with KIDNEY FAILURE (whether on dialysis or not)

Question 135

What is the purpose for using Erythropoiesis Stimulating Agents (epoetin and darbapoetin)?

Answer 135

To reduce dependence on transfusions

Question 136

What is the TARGET Hb at which time Erythropoietin Stimulating Agents (epoetin and darbapoetin) should be discontinued and why?

Answer 136

Hg ≥11 g/dL (because of higher rate of all-cause mortality, cardiovascular events and stroke

Question 137

What prophylactic medication is given to patients whom are undergoing myelosuppressive chemotherapy to reduce associated risk of febrile-NEUTROPENIA?

Answer 137

G-CSF (GRANULOCYTE-Colony Stimulating Factors) "granulocyte" is synonymous with "neutrophil"

Question 138

Immunosuppressive therapy (in Allogenic method to prevent GVHD - graft vs host disease) in conjunction with administration of Myeloablative doses of cytotoxic chemotherapy with total body irradiation followed by stem cell infusion?

Answer 138

Hematopoietic Stem Cell Transplantation (HSCT); allogenic (HLA-matched donor) is better tolerated than autologous (own cells used after massive doses of G-CSF are given)

Question 139

In what kind of Hematopoietic Stem Cell Transplantation (HSCT) technique is the risk of Opportunistic Bacterial, Viral and Fungal infections (Pneumocystis jirovecii pneumonia, Aspergillosis, HSV, CMV) highest?

Answer 139

ALLOGENIC HSCT (rather than autologous), because in order to prevent Graft Vs Host Disease, Immunosuppression MUST be used

Question 140

What cells do B-lymphocytes differentiate into that secrete a large number of ANTIBODIES (immunoglobulins)?

Answer 140

PLASMA cells (differentiated B-lymphocytes that secrete a large number of antibodies "immunoglobulins")

Question 141

MGUS, Amyloidosis, Multiple Myeloma and Waldenstom macroglobulinemia are all MALIGNANCIES caused the Abnormal Proliferation of these types of cells?

Answer 141

PLASMA cells (differentiated B-lymphocytes that secrete a large number of antibodies "immunoglobulins")

Question 142

Blacks, ≥70 yo, with a malignancy of PLASMA cells producing a Monoclonal (M) protein consisting of either IgG, IgA or IgD (heavy chains) and a K (kappa) or a Lambda light chain?

Answer 142

Multiple Myeloma

Question 143

Black, ≥70 yo with bone pain from lytic bone lesions, spinal cord compression with neurologic symptoms (bowel or bladder dysfunction) and sinus/pulmonary infections?

Answer 143

Multiple Myeloma

Question 144

What should be done for a patient with or without Multiple Myeloma that presents with bowel or bladder dysfunction attributed to spinal cord compression?

Answer 144

EMERGENCY!!

Question 145

ANEMIA with rouleaux formation, leukopenia, HYPERCALCEMIA and elevated CREATININE?

Answer 145

Multiple Myeloma

Question 146

Where are filtered monoclonal light-chains causing CAST Nephropathy found?

Answer 146

Multiple Myeloma

Question 147

How is Multiple Myeloma diagnosed?

Answer 147

Protein Electrophoresis, Immunofixation of Serum, 24-hour urine collection with identification of the M-protein (one heavy chain IgG, IgA or IgD with one light chain - kappa or lambda)

Question 148

Which are the ONLY two radiologic studies that can be used to detect Multiple Myeloma (lytic bone lesions)?

Answer 148

Myeloma BONE SURVEY (plain radiographs of the skeleton) or MRI

Question 149

What causes spinal cord compression with bowel and bladder dysfunction in patients with Multiple Myeloma and is an EMERGENCY?

Answer 149

Bone fragments from vertebral body compression fractures caused by lytic bone lesions or plasmacytomas

Question 150

What must be present on Bone Marrow biopsy for Multiple Myeloma to be diagnosed as "SYMPTOMATIC"?

Answer 150

≥10% clonal PLASMA cells, the PRESENCE of ANY amount of the M-protein and evidence of end-organ damage (lytic bone lesions)

Question 151

What type of Multiple Myeloma does a patient have who presents with ≥10% PLASMA cells on Bone Marrow biopsy, presence of ≥3g/dL of the M-protein but NO evidence of end-organ damage (lytic bone lesions)?

Answer 151

ASYMPTOMATIC (smoldering) Multiple Myeloma

Question 152

What can be used to predict the time to progression of ASYMPTOMATIC (smoldering) Multiple Myeloma to SYMPTOMATIC Multiple Myeloma?

Answer 152

Amount of PLAMSA cells and M-protein level

Question 153

What does a patient have if presents with NO SYMPTOMS but was found on Bone Marrow biopsy to have the M-protein although

Answer 153

Monoclonal Gammopathy of Undetermined Significance (MGUS)

Question 154

Which is the ONLY type of Multiple Myeloma that REQUIRES treatment?

Answer 154

SYMPTOMATIC Multiple Myeloma

Question 155

What does it mean in Monoclonal Gammopathy of Undetermined Significance (MGUS) when the M-protein found is ≥1.5 g/dL OR that the M-protein is not IgG but rather IgA or IgD?

Answer 155

Higher RISK of PROGRESSION (to Multiple Myeloma, Amyloidosis and Waldenstrom macroglobulinemia)

Question 156

What are the CORE meds that are used to treat Multiple Myeloma?

Answer 156

Thalidomide, Lenalidomide, Bortezomib and Melphalan

Question 157

Can Thalidomide and Lenalidomide be used in pregnant women?

Answer 157

NO WAY (teratogenic and thrombogenic)

Question 158

Thalidomide and Bortezomib, drugs used to treat Multiple Myeloma, have this significant side effect?

Answer 158

Peripheral Neuropathy

Question 159

Lenalidomide and Melphalan, drugs used to treat Multiple Myeloma, have this significant side effect?

Answer 159

Myelosuppression

Question 160

What should ALWAYS be considered PRIOR to starting chemotherapy agents for treatment of Multiple Myeloma?

Answer 160

Candidacy for AUTOLOGOUS Hematopoietic Stem Cell Transplantation (HSCT)

Question 161

What bisphosphonates are used in Multiple Myeloma with lytic bone lesions?

Answer 161

Pamidronate or Zoledronic acid

Question 162

Focal Segmental Glomerulosclerosis (FSGS), Acute Tubular Necrosis (ATN), osteonecrosis of the jaw with poor dentition or after dental procedure can occur with these meds, used in Multiple Myeloma for lytic bone lesions?

Answer 162

Pamidronate and Zoledronic acid (Bisphosphonates)

Question 163

How is Multiple Myeloma-related kidney injury treated?

Answer 163

By treating HYPRcalcemia and bisphosphonates as well as PLASMAPHERESIS (to reduce light-chains and real casts)

Question 164

How often should patients with ASYMPTOMATIC Multiple Myeloma be monitored for progression?

Answer 164

Every 3-6 months

Question 165

Prior to starting bisphosphonate therapy in a patient, what MUST be done in order to avoid bisphosphonate associated osteonecrosis of the jaw?

Answer 165

Dental evaluation

Question 166

What agents MUST be avoided in patients with Multiple Myeloma so as to prevent further kidney injury already caused by light-chain casts (which is an indication in itself to start aggressive Multiple Myeloma chemotherapy)?

Answer 166

NSAIDS, CT contrast agents, gadolinium, loop diuretics

Question 167

Incidentally found on SPEP (Serum Protein Electrophoresis), an M-protein (a heavy chain: IgG, IgA or IgD plus a light chain Kappa or Lambda)

Answer 167

Monoclonal Gammopathy of Undetermined Significance (MGUS)

Question 168

If a patient presents with symptoms suggestive of Multiple Myeloma, what should be tested first?

Answer 168

24-hour urine protein electrophoresis to look for the M-protein

Question 169

If a PLASMA cell disease is suspected in a symptomatic patient after finding the M-protein in the serum or urine, what should be the next diagnostic step?

Answer 169

Bone marrow biopsy to establish the need for treatment

Question 170

What is the risk of progression of MGUS to a clinically significant PLASMA cell disorder?

Answer 170

VERY low, about 1% per year

Question 171

Do most patients with MGUS progress to Multiple Myeloma?

Answer 171

NO!!

Question 172

Are most cases of Multiple Myeloma preceded by MGUS?

Answer 172

YES

Question 173

What can IgG, IgA (heavy chains) or a purely light chain (Kappa or Lambda) MGUS progress to?

Answer 173

ANY PLASMA cell disease (Multiple Myeloma, Amyloidosis)

Question 174

What can IgM MGUS progress to?

Answer 174

ONLY a B-cell non-Hodgkin lymphoma like Waldenstrom macroglobulinemia or amyloidosis

Question 175

How often should patients with MGUS be monitored for?

Answer 175

Every 6-12 months as well as Bone Mineral Density testing at baseline (because MGUS is associated with osteoporosis)

Question 176

Deposition of protein fibrils in tissues with subsequent end-organ damage is seen in what disease?

Answer 176

Amyloidosis

Question 177

Which protein fibrils are deposited in tissues with amyloidosis?

Answer 177

Lambda (most common) and Kappa LIGHT CHAINS

Question 178

Nephrotic proteinuria with worsening kidney function, Restrictive cardiomyopathy (when heart is involved) and hepatomegaly (when liver is involved), symmetric distal sensorimotor neuropathy, carpal tunnel syndrome, autonomic neuropathy and orthostatic hypotension can ALL be seen with this disease?

Answer 178

Amyloidosis

Question 179

Periorbital purpura and macroglossia are two characteristic findings seen in this disease?

Answer 179

Amyloidosis

Question 180

Finding of AMORPHOUS EOSINOPHILIC material from an abdominal FAT PAD aspirate or Bone Marrow biopsy with APPLE GREEN BIREFRINGENCE when stained with CONGO RED dye is characteristic of?

Answer 180

Amyloidosis (clonal light chains)

Question 181

If Amyloidosis is diagnosed, what should be checked next?

Answer 181

The HEART (ECG, ECHO (TTE) - interventricular septal hypertrophy, restrictive heart disease, "sparkling" myocardium)

Question 182

What is the treatment for Amyloidosis?

Answer 182

Dexamethasone + Melphalan

Question 183

What is a CURATIVE treatment for Amyloidosis used for young patients (

Answer 183

Autologous Hematopoietic Stem Cell Transplantation (HSCT)

Question 184

A B-cell LYMPHOMA consisting mainly of LYMPHOCYTES and PLASMA cells characterized by the production of monoclonal IgM antibodies found usually in elderly WHITE men?

Answer 184

Waldenstom macroglobulinemia

Question 185

Lymphadenopathy, Hepatosplenomegaly (as found in ALL), Hyperviscosity (as found in proliferative disorders like polycythemia vera), CNS symptoms, Retinal Hemorrhages,, peripheral sensorimotor neuropathy (as seen with Amyloidosis), mucosal bleeding (nasal) from platelet dysfunction and Anemia with rouleaux formation are all seen in?

Answer 185

Waldenstrom macroglobulinemia

Question 186

Bone Marrow biopsy demonstrating >10% of a lymphoplasmacytic lymphoma with IgM M-protein?

Answer 186

Waldenstrom macroglobulinemia

Question 187

Should ASYMPTOMATIC patients with Waldenstrom macroglobulinemia be treated?

Answer 187

NO!! (just as you would not treat an ASYMPTOMATIC patient with Multiple Myeloma)

Question 188

What should be done with a patient that manifests with HYPERviscosity syndrome with Waldenstrom macroglobulinemia?

Answer 188

EMERGENT Plasmapheresis!!

Question 189

How is Waldenstrom macroglobulinemia treated?

Answer 189

RITUXIMAB + (either an alkylating agent like chlorambucil or cyclophosphamide or nucleoside like fludarabine)

Question 190

Lymphadenopathy + Hepatosplenomegaly + Hyperviscosity=?

Answer 190

Waldenstrom macroglobulinemia

Question 191

What is the measure of the blood volume that is composed of erythrocytes (RBCs)?

Answer 191

Hematocrit (Hct)

Question 192

What portion of the CBC tells you about the SIZE of the erythrocytes (RBCs)?

Answer 192

MCV (Mean Corpuscular Volume)

Question 193

Why is the normal Hb level in men higher than that of women?

Answer 193

The eryhtropoietic effect of Androgens

Question 194

What is normal erythrocyte production controlled by?

Answer 194

Erythropoietin which is MADE by the KIDNEYS in response to HYPOXIA

Question 195

CBC with Microcytosis and Anisocytosis (RBCs of unequal size) with Poikilocytosis (RBCs of abnormal shape) are found in?

Answer 195

IRON-deficiency anemia

Question 196

CBC with Macrocytosis is found in?

Answer 196

Vitamin B12 (Cobalamin), Folate deficiency or Myelodysplasia

Question 197

Removal of what organ and disease of what other organ can cause target RBCs?

Answer 197

Splenectomy; Liver disease

Question 198

TTP, HUS, DIC has what type of RBC morphology?

Answer 198

Schistocytes (fragmented RBCs)

Question 199

Hemolysis, Hypoxia, Bone Marrow Stress has what type of RBC morphology?

Answer 199

Nucleated RBCs (reticulocytes)

Question 200

Teardrop RBCs are found in?

Answer 200

Myelofibrosis

Question 201

Bite Cells (RBCs) are found in what condition?

Answer 201

G6PD deficiency

Question 202

Burr Cells "echinocytes" (RBCs) are found in what condition?

Answer 202

Kidney disease

Question 203

Spur Cells "acanthocytes" (RBCs) are found in what condition?

Answer 203

SEVERE Liver Disease (can also see target cells as in splenectomy and Thalassemia)

Question 204

Spherocytes (RBCs) are found in what condition BESIDES hereditary spherocytosis?

Answer 204

WARM (antibodies) autoimmune Hemolytic Anemia

Question 205

Target Cells (RBCs) are typically found in what disease (aside from splenectomy and Liver disease?

Answer 205

Thalassemia

Question 206

Drug toxicities, Myelodysplasia and Alcohol can cause formation of what types of RBCs?

Answer 206

Macrocytic (as seen in cobalamin and folate deficiency)

Question 207

Why do pregnant, lactating women and normal child growth present with mild MICROcytic anemias?

Answer 207

Because those states have INCREASED iron utilization

Question 208

In MEN and NON-menstruating WOMEN, the most common reason for IRON Deficiency is?

Answer 208

GI blood loss

Question 209

Where is IRON absorbed in the intestine?

Answer 209

The SMALL bowel

Question 210

PICA (eating ice, clay, starch, paper) is caused by what?

Answer 210

IRON deficiency

Question 211

What nail findings are seen in a patient with SEVERE iron deficiency?

Answer 211

Spooning (koilonychia)

Question 212

Reduced serum IRON, Reduced serum FERRITIN, Reduced TRANSFERRIN saturation (Iron/TIBC), Increased TIBC indicate?

Answer 212

Iron deficiency

Question 213

Which IRON measure is an ACUTE Phase Reactant which even in the setting of IRON deficiency, can be NORMAL or ELEVATED if Inflammation is present?

Answer 213

FERRITIN

Question 214

Regardless of FERRITIN being an acute phase reactant, ABOVE what value and BELOW what value is IRON deficiency an ABSOLUTE or IMPOSSIBLE regardless of the presence of inflammation?

Answer 214

Ferritin >100, NO possibility of Iron deficiency | Ferritin

Question 215

What blood component is ELEVATED in IRON deficiency?

Answer 215

Platelets (thrombocytosis) due to blood loss

Question 216

What is the best IRON supplement to use for IRON deficiency?

Answer 216

Ferrous Sulfate, 325 mg po TID (66 mg iron per dose)

Question 217

Why should IRON supplements not be taken with meals, antacids, antibiotics?

Answer 217

Hinders proper absorption

Question 218

What is the BEST, most PRECISE measure of IRON deficiency?

Answer 218

Low HEPCIDIN levels (iron regulatory peptide made by the liver)

Question 219

In the acidic environment of the stomach, COBALAMIN (vitamin B12) is bound to R-binders found in the SALIVA and GASTRIC SECRETIONS. In the ALKALINE Small Bowel, the COBALAMIN is transferred from the R-binders to INTRINSIC FACTOR (IF) which is NECESSARY for absorption of the COBALAMIN in the ILEUM after which it is bound to TRANSCOBALAMIN and stored in the reticuloendothelial system in the LIVER

Answer 219

Normal Vitamin B12 absorption and processing

Question 220

What is the most COMMON cause of Cobalamin (vitamin B12) deficiency?

Answer 220

MALABSORPTION

Question 221

Ab-directed (autoimmune) destruction of gastric parietal cells (synthesize Intrinsic Factor -IF)?

Answer 221

Pernicious Anemia

Question 222

Pancreatic Insufficiency, Bacterial Overgrowth and Achlorhydria (low acid states) all cause COBALAMIN deficiency how?

Answer 222

By Malabsorption

Question 223

Loss of position or vibration sense with spastic ataxia, yellow skin from hemolysis, hallucination, dementia and psychosis can all be seen in this deficiency?

Answer 223

COBALAMIN (vitamin B12) deficiency

Question 224

Oval Macrocytes and HYPERsegmented neutrophils (>5 nuclear lobes) with thrombocytopenia and leukopenia are found in what deficiency?

Answer 224

COBALAMIN (vitamin B12) deficiency

Question 225

FOLATE deficiency is confirmed by elevation of WHAT analyte?

Answer 225

HOMOCYSTEINE ONLY

Question 226

COBALAMIN (vitamin B12) deficiency is confirmed by the elevation of what TWO analytes?

Answer 226

METHYLMALONIC ACID AND HOMOCYSTEINE

Question 227

Decreased HAPTOGLOBIN, ELEVATED serum LACTATE DEHYDROGENASE (LDH) and UNCONJUGATED Bilirubin levels and a LOW RETICULOCYTE suggest what?

Answer 227

Hemolysis (such as with COBALAMIN deficiency)

Question 228

Hemolysis with a LOW reticulocyte count suggests what?

Answer 228

RBC SYNTHESIS dysfunction (Bone marrow problems like myelofibrosis or decreased Iron, Cobalamin or Folate)

Question 229

Why is it SO important when a patient presents with Macrocytic Anemia, that the etiology of whether it is Cobalamin or Folate deficiency is DETERMINED?

Answer 229

Because the anemia WILL RESPOND to supplementation of FOLATE whether folate OR cobalamin was deficient, HOWEVER it may have been caused by COBALAMIN deficiency in which case the NEUROLOPSYCHIATRIC symptoms will WORSEN and become IRREVERSIBLE

Question 230

Is Methylmalonic acid increased in FOLATE deficiency?

Answer 230

NO!! only HOMOCYSTEINE is

Question 231

What is the BEST treatment of COBALAMIN deficiency?

Answer 231

ORAL supplementation

Question 232

Diseases with increased cell turnover such as PSORIASIS and HEMOLYTIC ANEMIAS (sickle cell anemia) as well as MALNUTRITION and ALCOHOL abuse can cause what important dietary deficiency?

Answer 232

FOLATE (very limited stores in body)

Question 233

Can the serum folate be normal in folate deficiency?

Answer 233

YES!! which is why you MUST measure the HOMOCYSTEINE level if you suspect a FOLATE deficiency

Question 234

What is "inflammatory anemia"?

Answer 234

What was called "anemia of chronic disease" in the past

Question 235

Why do states of inflammation cause anemia?

Answer 235

Inflammatory cytokines inhibit erythropoietin production, the response to erythropoietin as well as INCREASED production of the hepatic peptide HEPCIDIN which causes DECREASED IRON absoprtion and DECREASED release of iron from erythrocytes and macrophages

Question 236

Anemia with a Hb >8 g/dL, DECREASED reticulocyte levels (synthetic problem), normal to LOW (if chronic) serum IRON, LOW TIBC (no need to bind any new iron) and a HIGH FERRITIN level (

Answer 236

Inflammatory Anemia (formerly known as anemia of chronic disease)

Question 237

States of Inflammation as well as CHRONIC heart disease and DM cause this type of anemia?

Answer 237

Inflammatory Anemia (formerly known as anemia of chronic disease)

Question 238

Does Inflammatory Anemia need treatment OR IRON supplementation?

Answer 238

NO!!! treat the UNDERLYING disease

Question 239

Normochromic, Normocytic anemia with a REDUCED reticulocyte count (synthetic problem) with BURR Cells (echinocytes) in the ABSENCE of a Myelofibrotic disease?

Answer 239

Anemia caused by KIDNEY disease

Question 240

In the setting of KIDNEY disease, if a MICROCYTIC Anemia develops, what would be the cause and how would you check for it knowing that anemia of kidney disease is NORMOCYTIC?

Answer 240

GI blood loss; Check Erythropoietin levels

Question 241

What is the treatment of Anemia of Kidney disease?

Answer 241

Erythropoiesis Stimulating Agents - epoetin or darbapoetin (ONLY up to a Hb of 11!!) and INCREASE ONLY by 1 g/week otherwise risk thrombocytosis, HTN, STROKE and MI

Question 242

In patients with Anemia of Kidney disease who are also on DIALYSIS, what ELSE must they be treated with BESIDES Erythropoiesis Stimulating Agents - epoetin and darbapoetin?

Answer 242

IRON (IV)

Question 243

What should the Serum FERRITIN and IRON SATURATION be in ALL patients receiving Erythropoiesis Stimulating Agents - epoetin and darbapoetin?

Answer 243

Ferritin ≥100 µg/L and Iron Saturation ≥20%

Question 244

Hereditary Spherocytosis, ITP, TTP, G6PD deficiency, Thalassemia, Hemoglobinopathies, Warm Autoimmune/Cold Agglutinin, Paroxysmal Nocturnal Hemoglobinuria, Microangiopathic and Drug-Induced Anemias are what type of Anemias?

Answer 244

HEMOLYTIC anemias

Question 245

Jaundice (unconjugated - INDIRECT - bilirubinemia), pigmented gallstones, splenomegaly, elevated LDH, Haptoglobin and presence of Hemoglobinuria are common findings in this type of anemias?

Answer 245

HEMOLYTIC anemias

Question 246

With NORMAL Bone Marrow function and Erythropoietin production, what is the TYPICAL response to HEMOLYSIS?

Answer 246

Increased RETICULOCYTE level (bone marrow stress)

Question 247

If the etiology of a hemolytic anemia is not readily apparent, what should be the FIRST diagnostic test done to evaluate for an IMMUNE-MEDIATED hemolysis?

Answer 247

Coombs TEST (direct antiglobulin test)

Question 248

In what HEMOLYTIC Anemia is the Bone Marrow's ability to compensate also impaired leading to APLASTIC CRISIS?

Answer 248

Parvovirus B19

Question 249

Whatever the ETIOLOGY of HEMOLYTIC Anemia, BESIDES treating the UNDERLYING disease, what MUST be given to ALL patients with CHRONIC hemolytic anemia?

Answer 249

FOLIC ACID (1 mg/day)

Question 250

Mutations in the ANKYRIN protein leading SPECTRIN deficiency with loss of erythrocyte surface area leading to destabilized erythrocytes?

Answer 250

Hereditary Spherocytosis

Question 251

Severe neonatal jaundice and severe Parvovirus B19 infection-related apalstic-crisis is seen with this hematologic defect?

Answer 251

Hereditary Spherocytosis

Question 252

Pt has a family history of anemia, jaundice, splenomegaly and gallstones which would suggest what type of hereditary condition?

Answer 252

Hereditary Hemolytic anemia (hereditary Spherocytosis)

Question 253

Coombs test NEGATIVE (no anti-Ab's), OSMOTIC FRAGILITY TEST (in hypotonic saline) with 24-HOUR incubation is POSITIVE in a hemolytic anemia?

Answer 253

Hereditary Spherocytosis (a CONGENITAL not "autoimmune" hemolytic anemia)

Question 254

In a neonate born with jaundice, to test for Ab-MEDIATED HEMOLYSIS, to see if indeed FOREIGN antibodies already adherent to the newborn's RBCs, the NEWBORN's blood sample is incubated after adding anti-Human globulin (anti IgG and anti-complement). The anti-Human globulin BINDS to the NEWBORN's FOREIGN Ab's (IF THEY ARE THERE) that may have adhered to their RBCs causing them to clump up together in a process called "agglutination"

Answer 254

POSITIVE DIRECT Coombs Test (Direct anti-globulin test) can also be used for TRANSFUSION reactions and Drug-induced hemolysis

Question 255

To detect the presence of an RBC Ab in a patient, this test takes a sample of blood from the patient and only keeps the plasma which will have Ab's in it. The plasma is then incubated while adding SPECIFIC RBCs with already-present antigens on them which causes the Pt's Ab's to BIND to the added RBCs. Adding anti-Human globulin (anti IgG and anti-complement) causes them to clump up together in a process called "agglutination"

Answer 255

POSITIVE INDIRECT Coombs Test (Indirect anti-globulin test)

Question 256

Direct Coombs TEST result in Hereditary Spherocytosis?

Answer 256

NEGATIVE (a CONGENITAL not "autoimmune" hemolytic anemia)

Question 257

What is the DIRECT Coombs TEST used for?

Answer 257

To detect an AUTOIMMUNE Hemolytic Anemia

Question 258

What is the INDIRECT Coombs TEST used for?

Answer 258

To detect a potential MATERNAL blood incompatibility (Rh) with her fetus OR prior to giving a blood TRANSFUSION

Question 259

Patients with MODERATE symptomatic anemia (those who need treatment) with Hereditary Spherocytosis should be given what treatment?

Answer 259

Splenectomy (with vaccination for P. pneumoniae, H. influenza and Meningococcus)

Question 260

When pts are asplenic (functional or surgical), vaccinations against what must be given?

Answer 260

P. pneumoniae, H. influenza and Meningococcus

Question 261

An X-linked erythrocyte enzyme defect most commonly found in BLACK MEN that has been found, when heterozygous, to protect against Plasmodium falciparum malaria?

Answer 261

G6PD deficiency

Question 262

This erythrocyte enzyme deficiency results in the erythrocyte not being able to make NADPH leading to episodic hemolysis in response to oxidant stressors (infection, drugs - dapsone, TMP-SMX, nitrofurantoin and FAVA beens)?

Answer 262

G6PD deficiency

Question 263

What is seen on a peripheral smear of a patient's blood with G6PD deficiency?

Answer 263

BITE cells and Heinz bodies (denatured, oxidized Hb)

Question 264

Checking G6PD levels at the same time as a hemolytic event occurred would result as NORMAL because ELEVATED G6PD would be found in young reticulocytes who have not yet matured. When would be an optimal time AFTER an acute event to check G6PD levels?

Answer 264

SEVERAL MONTHS after

Question 265

How is a G6PD acute hemolytic crisis treated?

Answer 265

Supportive with discontinuation of the agent that caused the hemolysis or treatment of the infection that did

Question 266

Microcytic, hypochromic erythrocytes and TARGET CELLS with IRON overload in the ABSENCE of transfusions are seen on peripheral blood smear in?

Answer 266

BOTH alpha and beta Thalassemia

Question 267

African Americans and Middle Eastern people tend to have which Thalassemia more commonly?

Answer 267

alpha-Thalassemia

Question 268

Mediterraneans, Indians, Asians and Pakistanis tend to have which Thalassemia more commonly?

Answer 268

beta-Thalassemia

Question 269

How many gene deletions occur in the alpha-Thalassemia SILENT CARRIER (normal medically and hematologically)?

Answer 269

ONE deletion of 4 (-a/aa)

Question 270

How many gene deletions occur in the alpha-Thalassemia TRAIT (or "MINOR") (mild anemia, microcytosis, hypochromia and target cells with NORMAL Hb electrophoresis)?

Answer 270

TWO deletions of 4 (-a/-a) or (--/aa)

Question 271

How can you distinguish IRON deficiency anemia from alpha-Thalassemia Minor (trait)?

Answer 271

By testing for RDW (RBC Distribution Width) which is ELEVATED in IRON deficiency but NORMAL in Thalassemia

Question 272

What is the RBC Distribution Width (RDW)?

Answer 272

The variation in volume (size) between different RBCs in the same sample

Question 273

Thalassemia associated with CHROMOSOME 16?

Answer 273

alpha-Thalassemia

Question 274

Thalassemia associated with CHROMOSOME 11?

Answer 274

beta-Thaassemia

Question 275

Do alpha-Thalassemia SILENT CARRIER or alpha-Thalassemia TRAIT (MINOR) require treatment?

Answer 275

NO

Question 276

What is Hb-H disease?

Answer 276

alpha-Thalassemia with THREE gene deletions (--/-a)

Question 277

Which alpha-Thalassemia is associated with Splenomegaly, SEVERE anemia, HF and hypoxia as well as being readily identified on Hb electrophoresis?

Answer 277

Hemoglobin-H (Hb-H) Thalassemia

Question 278

What is Hb-BARTS?

Answer 278

Homozygous inheritance of a DOUBLE gene deletion where ALL four genes are deleted in alpha-Thalassemia (--/--) causing Hydrops Fetalis (death in utero)

Question 279

How is Hb-H (alpha-Thalassemia with THREE gene deletions) treated?

Answer 279

Intermittent TRANSFUSIONS

Question 280

How is beta-Thalassemia different than alpha-Thalassemia?

Answer 280

There are no gene "DELETIONS" just over or under EXPRESSION of the beta-globin gene

Question 281

Mildly-DECREASED expression of a SINGLE (of two) beta-globin gene on Hb results in what?

Answer 281

beta-Thalassemia TRAIT (mild anemia, microcytosis, hypochromia and target cells) - no treatment needed

Question 282

What is the Mentzer Index?

Answer 282

MCV/RBC count >13 is associated with beta-Thalassemia

Question 283

How can beta-Thalassemia TRAIT be diagnosed in the laboratory?

Answer 283

Hb electrophoresis (shows increased Hb A2 and Hb F)

Question 284

What is beta-Thalassemia MAJOR?

Answer 284

(Cooley anemia) beta-Thalassemia with almost completely ABSENT synthesis of the beta-globin chain

Question 285

beta-Thalassemia associated with SEVERE anemia, growth retardation, skeletal complications and iron overload?

Answer 285

beta-Thalassemia MAJOR (Cooley anemia)

Question 286

How is a patient with beta-Thalassemia MAJOR (Cooley anemia) treated?

Answer 286

Life-long blood transfusions WITH iron-CHELATION therapy and SPLENECTOMY to reduce transfusions followed by appropriate vaccination (P. pneumoniae, H. influenza, Meningococcus)

Question 287

What treatment for beta-Thalassemia MAJOR (Cooley anemia) is potentially CURATIVE?

Answer 287

Allogenic Hematopoietic Stem Cell Transplantation (HSCT)

Question 288

What is beta-Thalassemia Intermedia?

Answer 288

Decreased but not absent expression of the beta-globin gene leading to a phenotype somewhere in BETWEEN beta-Thalassemia TRAIT and beta-Thalassemia MAJOR

Question 289

Do you treat beta-Thalassemia TRAIT?

Answer 289

NO

Question 290

Do you treat beta-Thalassemia Intermedia?

Answer 290

YES, intermittent transfusions and iron chelation therapy

Question 291

9% of BLACKS have Hb-AS, what is that?

Answer 291

Sickle Cell TRAIT (heterozygous for Hb-S)

Question 292

What is Hb-A (a2b2)?

Answer 292

Normal adult hemoglobin

Question 293

Although considered a benign condition, this Sickle Cell Hb expression has been associated with hematuria, renal medullary carcinoma, risk of splenic rupture at high-altitudes, venous thromboembolism and sudden death during extreme conditions?

Answer 293

Hb(AS) or "Sickle Cell TRAIT"

Question 294

What is screening recommended for all student athletes prior to participation in athletic activities?

Answer 294

Screening for Sickle Cell TRAIT (Hb-AS) and if positive recommend Optimal HYDRATION during strenuous activity

Question 295

What is Sickle Cell DISEASE?

Answer 295

Patients with Sickle Cell GENOTYPES associated with HEMOLYSIS and Vasoocclusive crisis (homozygous Sickle Cell Anemia - Hb(SS), Sickle-beta Thalassemia - Hb(Sß*) and Hb(Sߺ) and Hemoglobin SC Disease - Hb(SC)

Question 296

Hb(SS) - homozygous Sickle Cell Anemia and Sickle-beta Thalassemia Hb(Sߺ) have what type of presentation?

Answer 296

Reduced life expectancy, moderate to severe anemia and frequent pain crises

Question 297

Hb(SC) and Hb(Sß*) have what type of presentation?

Answer 297

Less severe anemia and Less frequent vasoocclusive crises however still with ocular complications and bony infarcts

Question 298

What is the BEST treatment for a patient with Sickle Cell DISEASE [Hb(SS), Hb(Sߺ), Hb(Sß*), Hb(SC)] which has shown to DECREASE mortality and is used in patients with recurrent painful episodes, acute chest syndrome and symptomatic anemia?

Answer 298

Hydroxyurea (increases formation of Hb-F and breaks down cells that are about to sickle)

Question 299

Can a patient with Sickle Cell Disease use Hydroxyurea if they are planning to become pregnant?

Answer 299

NO!!

Question 300

How is an UNCOMPLICATED painful Sickle Cell episode treated?

Answer 300

HYDRATION, ANALGESIA, Incentive Spirometry (to AVOID Acute Chest Syndrome) - discharge on hydroxyurea to decrease painful episodes and hospitalizations

Question 301

What are the analgesic agents of CHOICE when treating Sickle Cell pain?

Answer 301

MORPHINE and Hydromorphone

Question 302

Stroke, Acute Chest Syndrome, Pulmonary HTN, Priapism, Infection (from functional asplenia), Anemia are all manifestations of what?

Answer 302

Sickle Cell DISEASE [Hb(SS), Hb(Sߺ), Hb(Sß*), Hb(SC)]

Question 303

How is a Sickle Cell Disease patient treated for STROKE?

Answer 303

Erythrocyte EXCHANGE Transfusion (reduce Hb-S

Question 304

A patient with Sickle Cell DISEASE p/w ACUTE hypoxia, NEW infiltrate on an ENTIRE segment on CXR caused either by an INFECTION, Bone Marrow FAT embolus or a Pulmonary Infarction?

Answer 304

Acute Chest Syndrome

Question 305

How is Acute Chest Syndrome treated?

Answer 305

Broad Spectrum ANTIBIOTICS, OXYGEN, PAIN Meds, Incentive SPIROMETRY to reduce atelectasis, Transfusion if needed and avoidance of overhydration

Question 306

What is done EMERGENTLY for a patient with Sickle Cell DISEASE who presents either with a STROKE or Acute Chest Syndrome WITH SEVERE/PROGRESSIVE Hypoxia?

Answer 306

Erythrocyte EXCHANGE Transfusion

Question 307

What medication given to patients with Sickle Cell DISEASE has been shown to decrease the frequency of ACUTE CHEST SYNDROME?

Answer 307

Hydroxyurea

Question 308

A complication of Sickle Cell DISEASE that requires EMERGENT Urological Consultation for decompression if >2 hours with IVF, Pain management and Oxygenation?

Answer 308

Priapism

Question 309

What infections are Sickle Cell DISEASE patients at higher risk for and require vaccinations?

Answer 309

H. influenza, P. pneumoniae, Meningococcus, Hep B and Influenza virus (yearly)

Question 310

What are the three possibilities of a SEVERE ACUTE ANEMIA in a patient with Sickle Cell DISEASE?

Answer 310

Parvovirus B19 infection, Splenic/Hepatic sequestration or superimposed G6PD deficiency

Question 311

What are the ONLY indications for Erythrocyte Transfusion in patients with Sickle Cell DISEASE?

Answer 311

Symptomatic Anemia (Hb-SS, Hb-Sߺ), Stroke, Acute Chest Syndrome and Surgical Intervention

Question 312

Transfusion with Leukoreduced HbS-NEGATIVE, phenotypically matched for E, C and Kell antigens and alloantibodies Erythrocytes are typically used for what patients?

Answer 312

Those with Sickle Cell DISEASE who REQUIRE transfusion

Question 313

Which are the two most commonly implicated antibodies in Autoimmune Hemolytic Anemia (AIHA)?

Answer 313

IgG (80%) and IgM (20%)

Question 314

Abnormal IgG antibodies binding to Erythrocyte Rh antigens at 37ºC causing the cells to be Hemolysed by the SPLEEN after alteration into SPHEROCYTES by splenic macrophages with resultant MILD SPLENOMEGALY, anemia, jaundice and obviously a POSITIVE DIRECT Coombs Test?

Answer 314

WARM Autoimmune Hemolytic Anemia (37ºC) - POSITIVE Coombs for IgG and negative or weakly positive for C3 (complement)

Question 315

How do you treat WARM Autoimmune Hemolytic Anemia?

Answer 315

CORTICOSTEROIDS (prednisone) and if not responsive, RITUXIMAB or SPLENECTOMY

Question 316

Coombs test is positive for IgG in what type of Autoimmune Hemolytic Anemia?

Answer 316

WARM

Question 317

Coombs test is positive for ONLY complement C3 in what type of Autoimmune Hemolytic Anemia?

Answer 317

COLD

Question 318

Anemia worsened at cold temperatures (

Answer 318

Cold Agglutinin Disease (Cold Autoimmune Hemolytic Anemia)

Question 319

Cold Agglutinin Disease (Cold Autoimmune Hemolytic Anemia) has been seen after INFECTIONS with what agents and results in a NEGATIVE Coombs Test for IgG, but a POSITIVE Coombs Test for complement (C3)?

Answer 319

Mycoplasma and EBV

Question 320

What is the first line RECOMMENDATION for patients with Cold Agglutinin Disease (Cold Autoimmune Hemolytic Anemia)?

Answer 320

Avoidance of Cold Weather and wearing warm clothing

Question 321

What is the medical treatment for Cold Agglutinin Disease (Cold Autoimmune Hemolytic Anemia)?

Answer 321

RITUXIMAB or Chlorambucil or Cycophosphamide (corticosteroids and splenectomy are ineffective)

Question 322

For which of the Autoimmune Hemolytic Anemias are CORTICOSTEROIDS and SPLENECTOMY recommended?

Answer 322

WARM Autoimmune Hemolytic Anemia

Question 323

What should be avoided in ALL patients with Autoimmune Hemolytic Anemias unless ABSOLUTELY necessary?

Answer 323

Blood Transfusions (due to acute or delayed hemolytic transfusion reactions)

Question 324

Autoimmune Hemolytic Anemias that are caused by medications, typically occur DAYS to WEEKS after taking the medication and are most commonly associated with what medications?

Answer 324

Cephalosporins (2nd and 3rd gen)

Question 325

What does the Direct Coombs Test show in DRUG-Induced Autoimmune Hemolytic Anemia?

Answer 325

Can be positive for IgG, C3, both or negative

Question 326

How is DRUG-Induced Autoimmune Hemolytic Anemia treated?

Answer 326

By DISCONTINUATION of the DRUG (can be prolonged if FLUDARABINE was the drug)

Question 327

What is the function of HAPTOGLOBIN and why is it DECREASED in HEMOLYTIC anemias?

Answer 327

Haptoglobin is found in the plasma and it BINDS free Hb that has been released by lysed RBCs (saves the Hb) therefore in the setting of a hemolytic anemia, its numbers will decrease as it binds as much of the free Hb in the plasma as it can

Question 328

Why is LDH significant in hemolytic anemia?

Answer 328

Because it is found in LARGE amounts in RBCs and when these are lysed, it is released therefore raising its numbers significantly

Question 329

Schistocytes and "helmet cells" with LOW HAPTOGLOBIN and HIGH LDH are found in what conditions?

Answer 329

Microangiopathic Hemolytic Anemias

Question 330

Turbulent flow around mechanical heart valves, balloon pumps, ventricular assist devices and aneurysms, TTP, HUS and DIC with schistocytes and helmet cells are all seen in this type of Anemia?

Answer 330

Microangiopathic Hemolytic Anemia

Question 331

In ANY patient with schistocytes in the blood, what should ALWAYS be checked for?

Answer 331

TTP (Thrombotic Thrombocytopenic Purpura),HUS and DIC (only if there is an associated coagulopathy - low platelets)

Question 332

How are TTP and HUS treated?

Answer 332

PLASMA EXCHANGE

Question 333

How is DIC treated?

Answer 333

Prompt treatment of UNDERLYING infection or OBSTETRIC emergencies and OFFENDING DRUG Discontinuation

Question 334

FEVER + NEUROLOGIC SYMPTOMS + HEMOLYSIS =?

Answer 334

TTP - plasma exchange

Question 335

E.coli + DIARRHEA + HEMOLYSIS =?

Answer 335

HUS - plasma exchange

Question 336

Pt presents with hemolytic anemia, PANcytopenia, unprovoked thrombosis, mutation in PIG-A gene resulting in a decrease or ABSENCE of an important erythrocyte anchoring protein where hemolysis occurs by absence of CD55 and CD59 regulating factors?

Answer 336

Paroxysmal Nocturnal Hemoglobinuria (PNH)

Question 337

Detection of CD55 and CD59 deficiency on FLOW CYTOMETRY on the surface of erythrocytes and leukocytes is diagnostic for?

Answer 337

Paroxysmal Nocturnal Hemoglobinuria

Question 338

Pancytopenia, atypical-site thrombosis such as in the mesenteric or cerebral circulation with hemolysis?

Answer 338

Paroxysmal Nocturnal Hemoglobinuria (PNH)

Question 339

How is Paroxysmal Nocturnal Hemoglobinuria treated?

Answer 339

AGGRESSIVE ANTICOAGULATION and ECULIZUMAB (monoclonal Ab against the C5 terminal complement component)

Question 340

What is of UTMOST importance when treating a patient with Paroxysmal Nocturnal Hemoglobinuria (PNH) with ECULIZUMAB?

Answer 340

Vaccination against Meningococcus due to HIGH risk of Neisserial infection with this drug

Question 341

In patients with Paroxysmal Nocturnal Hemoglobinuria (PNH) who do not respond to ECULIZUMAB, have unresponsive hemolysis and severe marrow failure, what can be done?

Answer 341

Immunosuppressive therapy or Allogenic Bone Marrow Transplantation

Question 342

AGGRESSIVE Anticoagulation and Eculizumab are the treatment for what disease?

Answer 342

Paroxysmal Nocturnal Hemoglobinuria (PNH)

Question 343

What is the most common cause of INFECTIOUS Hemolytic anemia caused by INTRAVASCULAR hemolysis and by the splenic removal of infected erythrocytes?

Answer 343

Malaria

Question 344

In patients with FUNCTIONAL ASPLENIA or previous surgical splenectomy, this TICK-borne infectious agent found in CAPE COD, NANTUCKET or NORTH CALIFORNIA can result in a SEVERE hemolytic anemia?

Answer 344

Babesiosis

Question 345

What diarrheal illness can cause hemolysis due to its toxin?

Answer 345

Clostridium sepsis

Question 346

In South America (Brazil, Peru, Argentina, Venezuela, etc.), a SAND FLY can cause this disease that can result in SEVERE, RAPID, life-threatening hemolysis?

Answer 346

Bartonellosis (Oroya fever)

Question 347

What is the principal molecule that regulates IRON homeostasis and is made in the liver?

Answer 347

HEPCIDIN (decreases iron absorption from the intestine and inhibits iron release from macrophages)

Question 348

What special molecule made in the liver that regulates IRON homeostasis is LOW in HEMOCHROMATOSIS thereby causing a much higher intestinal absorption of iron? What gene is affected?

Answer 348

Hepcidin; HFE gene

Question 349

Cardiomyopathy, Arrhythmias, ELEVATED LFTs, Cirrhosis and HCC, DM, Arthropathy and BRONZING of the skin?

Answer 349

Hemochromatosis

Question 350

What is the best TEST to evaluate for HEMOCHROMATOSIS other than genetic testing for HFE gene mutation?

Answer 350

Serum TRANSFERRIN >60% (or Ferritin >1000 IF NO INFLAMMATORY STATE), can do liver biopsy if needed

Question 351

How is HEMOCHOMATOSIS treated?

Answer 351

Like Polycythemia Vera, by PHLEBOTOMY, maintaining serum FERRITIN levels

Question 352

Why is it important to diagnose HEMOCHROMATOSIS early?

Answer 352

Because can prevent cardiac, liver, DM pathology if treatment starts early, once disease affects these organs, it will not be reversible

Question 353

In patients with IRON overload from frequent transfusions, what is used to treat?

Answer 353

DEFERASIROX (NOT phlebotomy)

Question 354

What is the BEST choice of blood transfusion type to be given in an EMERGENCY when no information is known abut the recipient's blood type?

Answer 354

Type O-negative (negative refers to the Rh(D) antigen)

Question 355

What is the ONLY true indication for erythrocyte transfusion?

Answer 355

To INCREASE the OXYGEN-carrying capacity of blood

Question 356

What should be the transfusion Hb threshold in a CRITICALLY ILL patient who is NOT actively bleeding and WITHOUT cardiac compromise?

Answer 356

7 g/dL (if cardiac compromise, 10 g/dL)

Question 357

What does LEUKOREDUCTION do to a blood transfusion?

Answer 357

Removes leukocytes decreasing HLA alloimmunization (alloantibodies), reduces FEBRILE non-hemolytic transfusion reactions and decreases CMV transmission

Question 358

For planned intracranial surgery, what must the platelet count be above prior to warranting a platelet transfusion?

Answer 358

>100,000/µL

Question 359

What is the combination of Factor VIII, Fibrinogen, von Willebrand Factor (vWF) and Factor XIII called?

Answer 359

Cryoprecipitate (a transfusion for hypofibrinogenemia, Factor XIII deficiency and Hemophilia A or von Willebrand disease)

Question 360

What patients warrant use of Erythropoietin Stimulating Agents (epoetin and darbaportin)?

Answer 360

Those with kidney disease associated anemia and those with malignancy associated anemia

Question 361

What is the danger with the use of Erythropoietin Stimulating Agents (epoetin and darbapoetin) and what should be the Hb limit when used?

Answer 361

High risk for thrombosis; limit use to a Hb of 11 g/dL

Question 362

When a patient receives a PLATELET transfusion and becomes alloimunized to Human Leukocyte platelet Antigens (HLA), especially HLA-I Ag, what happens?

Answer 362

They become REFRACTORY to future PLATELET transfusions, requiring HLA MATCHING

Question 363

ONE single-donor platelet unit is the SAME as SIX pooled random-donor units and would provide how many platelets?

Answer 363

20,000-30,000/µL

Question 364

Platelet Transfusions carry a higher risk of bacterial infection and sepsis than compared with other blood products (kept at room temperature), what is the most common infectious agent?

Answer 364

Staph. aureus

Question 365

In a patient with Leukemia, without other risk factors and no active bleeding, at what level of platelets would a platelet transfusion be recommended?

Answer 365

≤10,000/µL

Question 366

What is the condition called when a patient receives a standard ONE single-donor unit of platelets or the equivalent of SIX units of pooled random-donor platelets and their platelet count, when checked ONE HOUR after transfusion only rises by ~10,000/µL?

Answer 366

Platelet Transfusion Refractoriness

Question 367

What are the most common causes of Platelet Transfusion Refractoriness?

Answer 367

Fever, DIC, Amphotericin B (antifungal medication) and patient Alloimmunization to HLA platelet antigens

Question 368

What type of platelet transfusions can be given if platelet transfusion refractoriness is found in a patient?

Answer 368

Single-donor, ABO-matched platelets OR HLA-matched platelets or "Cross-Matched" compatible platelets

Question 369

What is FFP and what is it used for?

Answer 369

Plasma containing ALL clotting factors, it is used for WARFARIN reversal in active-bleeding patients, TTP, Dilutional Coagulopathy during a massive transfusion and in patients with SEVERAL factor deficiencies (DIC, liver disease)

Question 370

How do you now HOW many units of FFP to give a patient?

Answer 370

One unit of FFP is 200-300 mL and the effective dose is 10-15 mL/kg (therefore a 70 kg pt should receive ~4 units

Question 371

What is a possible pulmonary complication seen with transfusions of FFP?

Answer 371

Transfusion-Related Acute Lung Injury (TRALI)

Question 372

What can transfusing erythrocytes with ABO incompatibility cause?

Answer 372

Acute Hemolytic Transfusion Reaction (clerical error, mislabeling)

Question 373

Pt receives a blood transfusion and develops HYPOtension, Kidney failure, Fever, Pain at INFUSION SITE, DIC?

Answer 373

Acute Hemolytic Transfusion Reaction

Question 374

What is the treatment for the ABO-caused Acute Hemolytic Transfusion Reaction?

Answer 374

STOP transfusion, send specimen to blood bank to test for incompatibility and hemolysis, give IVFs, Vasopressor support and Mannitol (diuretic)

Question 375

Pt presents with Anemia, Jaundice, Fever, worsening pain crisis in Sickle Cell DISEASE after receiving a blood transfusion 5-10 DAYS prior?

Answer 375

DELAYED Hemolytic Transfusion Reaction (amnestic response of a pre-formed erythrocyte alloantibody after exposure to an erythrocyte antigen OUTSIDE of the ABO system

Question 376

What should be done for a patient with DELAYED Hemolytic Transfusion Reaction (5-10 days after a transfusion)?

Answer 376

Repeat Type & Screen will identify the new alloantibody and supportive treatment only with MINIMIZED subsequent transfusions

Question 377

Dyspnea, cough, tachycardia, cyanosis, edema and chest tightness in a very young or very old patient after receiving a transfusion is caused by?

Answer 377

Fluid overload from Transfusion-Associated Circulatory Overload

Question 378

What is the MOST common cause of Transfusion-Related Death?

Answer 378

Transfusion Related Acute Lung Injury (TRALI)

Question 379

Antibodies in a transfusion donor's PLASMA directed against a recipient's NEUTROPHIL antigens causing leukocyte sequestration in the lungs and capillary LEAK?

Answer 379

Transfusion Related Acute Lung Injury (TRALI)

Question 380

Within 6-HOURS of a transfusion, a patient begins having HYPOXIA and DYSPNEA resembling pulmonary edema with fever and HYPOtension and BILATERAL lung infiltrates requiring INTUBATION and supportive treatment?

Answer 380

Transfusion Related Acute Lung Injury (TRALI)

Question 381

What should be done as soon as FEVER develops in a patient receiving a blood transfusion?

Answer 381

STOP transfusion and EXCLUDE a possible Acute Hemolytic Transfusion Reaction (ABO incompatibility) and continue transfusion treating fever, which can occur and is benign (may benefit from future leukoreduced transfusions)

Question 382

What patients are susceptible to ANAPHYLACTIC reactions during blood transfusions?

Answer 382

Those with IgA deficiency and have anti-IgA Ab's (subsequent transfusions should be washed of plasma proteins)

Question 383

What are common, benign reactions during transfusions that are cause by donor plasma proteins?

Answer 383

Fever and Urticaria (MUST stop after fever develops and rule out ABO incompatibility)

Question 384

What patients are at risk for FATAL Transfusion-related Graft vs HOST Disease (GVHD)?

Answer 384

Immunocompromised/suppressed, those receiving Hematopoietic Stem Cell Transplantation (HSCT) and those receiving blood from 1st degree relatives

Question 385

How is Transfusion-related Graft vs Host Disease (GVHD) eliminated for susceptible patients (immunocompromised/suppressed, Hematopoietic Stem Cell Transplantation-HSCT and those receiving blood from 1st degree relatives)?

Answer 385

By receiving IRRADIATED blood products

Question 386

Separation of WHOLE blood into its components with TREATMENT or REMOVAL of affected component and RETURN remaining blood products is called APHERESIS and is used in these conditions?

Answer 386

Sickle Cell DISEASE p/w ACUTE Chest Syndrome, Stroke or Multi-organ Failure, Malaria and Babesia

Question 387

The removal of the patient's plasma and replacement either with DONOR plasma or an albumin/saline mixture is called PLASMA EXCHANGE and is used in these conditions?

Answer 387

Cryoglobulinemia, TTP, Acute Inflammatory Demyelinating Polyneuropathy (Guillain-Barre), Chronic Inflammatory Demyelinating Polyneuropathy, Myesthenia Gravis, Goodpasture Syndrome, Granulomatosis with Polyangiitis (Wegener Granulomatosis) - related RPGN, Renal Transplant Ab-mediated Rejection, IgM-related Polyneuropathy, Lambert-Eaton Syndrome (paraneoplastic disease associated with SMALL CELL LUNG CA that acts like Myesthenia Gravis), MS and Myeloma Cast Nephropathy.

Question 388

What should be monitored in patients undergoing Therapeutic Apheresis (Sickle Cell Disease with acute chest syndrome, stroke or multi-organ failure, babesia or malaria)?

Answer 388

Calcium levels (can cause severe hypocalcemia) and HYPOtension (discontinue ACE-I prior to procedure)

Question 389

A patient's history of bleeding into muscles and joints suggests what type of disorder?

Answer 389

Disorder of the clotting factors themselves

Question 390

A patient's history of MUCOSAL bleeding suggests what type of disorder?

Answer 390

Disorder of primary hemostasis (formation of a platelet plug), thrombocytopenia or a qualitative platelet disorder

Question 391

10%-30% of women who report excessive and prolonged gynecological/menstrual bleeding have what?

Answer 391

Underlying BLEEDING Disorder

Question 392

What are petechiae (small red spots on skin indicative of extravasated erythrocytes) indicative of?

Answer 392

Thrombocytopenia

Question 393

Peri-follicular hemorrhages and cork-screw hairs as well as bleeding gums are seen in this vitamin deficiency?

Answer 393

Vitamin C deficiency (ascorbic acid) - SCURVY

Question 394

Enlarged tongue (macroglossia), Carpal Tunnel Syndrome, periorbital purpura indicate?

Answer 394

Amyloidosis

Question 395

Telangiectasias on lips and fingertips?

Answer 395

Hereditary Hemorrhagic Telangiectasia

Question 396

Acquired deficiency of Factor VII (Vitamin K deficiency, Liver disease, DIC or Warfarin) results in prolongation of which clotting assay?

Answer 396

PT

Question 397

If aPTT is elevated by itself and PT is not, what could MOST COMMONLY cause this?

Answer 397

Lupus inhibitor or Hemophilia

Question 398

What agents prolong the thrombin Clotting Time?

Answer 398

Any thrombin inhibitor (heparin, lepiRUDIN, argaTROBAN, dabiGATRAN) or Fibrinogen abnormality

Question 399

If aPTT is prolonged, what study is used to evaluate whether the prolongation was due to a clotting factor inhibitor such as the Lupus inhibitor or due to a clotting factor deficiency as occurs in Hemophilia?

Answer 399

Mixing Study: prolonged aPTT result: 1. mix pt's PLASMA with normal PLASMA (1:1) repeat aPTT 2. If aPTT is now NORMAL = Factor Deficiency 3. If aPTT is INCOMPLETELY corrected = Inhibitor

Question 400

So, after a prolonged aPTT, a mixing study remains OUTSIDE the normal range (only partially corrected) what is the cause of the prolonged aPTT?

Answer 400

The presence of an INHIBITING factor (Lupus inhibitor)

Question 401

What does the D-dimer assay measure?

Answer 401

The breakdown products of the fibrin clot

Question 402

Bleeding into muscles and joints suggests what type of bleeding disorder?

Answer 402

Disorder of the clotting factors

Question 403

Bleeding from mucosal surfaces suggests what type of bleeding disorder?

Answer 403

Disorder of platelets (thrombocytopenia or platelet defects) and platelet adhesion (von Willebrand Factor)

Question 404

Clotting Factor VIII deficiency results in what disease?

Answer 404

Hemophilia A (X-linked disorder)

Question 405

Clotting Factor IX deficiency results in what disease?

Answer 405

Hemophilia B (X-linked disorder)

Question 406

What is the severity of Hemophilia A and B determined by?

Answer 406

How much of a factor deficiency they have

Question 407

Recurrent HAMARTHROSES (bleeding into joints) and bleeding into MUSCLES resulting in chronic degenerative joint disease, CNS hemorrhage and post-traumatic or post-surgical bleeding?

Answer 407

Hemophilia

Question 408

What is the treatment of Hemophilia (A or B)?

Answer 408

Factor VIII and IX concentrates as well as Desmopressin (vasopressing analogue inhibiting urine production)

Question 409

What medications are absolutely CONTRAINDICATED in patient with Hemophilia?

Answer 409

ASPIRIN and NSAIDS

Question 410

What is the MOST COMMON Inherited Bleeding Disorder?

Answer 410

von Willebrand Disease (vWD) - low levels of the von Willebrand Factor (vWF)

Question 411

What is the function of the von Willebrand Factor (vWF)?

Answer 411

It PROTECTS Factor VIII from degradation

Question 412

Mucocutaneous bleeding, significant menorrhagia, endometriosis and postpartum hemorrhage and a slightly prolonged aPTT are seen in this disease?

Answer 412

von Willebrand Disease (vWD)

Question 413

How is MILD von Willebrand Disease treated? moderate-severe?

Answer 413

Desmopressin; Infusions with vWF-containing Factor VIII concentrates

Question 414

The presence of low levels of the von Willebrand Factor (vWF) suggests what disease?

Answer 414

von Willebrand Disease (vWF prevents degradation of Factor VIII)

Question 415

What coagulation assay is PROLONGED when there is a Factor VII deficiency, DIC, Liver Disease or Vitamin K deficiency?

Answer 415

PT

Question 416

What DRUGS PROLONG both PT and aPTT?

Answer 416

Heparin and Direct Thrombin Inhibitors (argaTROBAN, dabiGRATAN, lepiRUDIN, bivaliRUDIN)

Question 417

How do you reverse anticoagulation with UNFRACTIONATED Heparin?

Answer 417

Protamine Sulfate

Question 418

What do you give a patient in DIC besides antibiotics and treating underlying disease?

Answer 418

FFP, Platelets, Cryoprecipitate (EVERYTHING)

Question 419

What are the PT and aPTT in von Willebrand Disease, Factor XIII deficiency, Platelet deficiency, LMWH?

Answer 419

NORMAL

Question 420

Factor II+X, II+VII+IX+X (liver disease) V, V+VIII, X, hypofibrinogenemia/fibrinolysis (with tPA), Lupus Inhibitor, Vitamin K deficiency or antagonists (WARFARIN) PROLONG which coagulation assays?

Answer 420

PT and aPTT

Question 421

What is the PT and aPTT with unfractionated Heparin? LMWH?

Answer 421

unfractionated Heparin: PROLONGED (Both PT and aPTT) | LMWH: NORMAL (Both PT and aPTT)

Question 422

When a patient's PT and aPTT are BOTH prolonged or there is suspected unfractionated Heparin contamination (or any direct thrombin inhibitor) of the sample, what test can be used?

Answer 422

Clotting Time (PROLONGED)

Question 423

The postpartum state, a malignancy or autoimmune conditions can cause this disease which occurs due to a clotting factor deficiency?

Answer 423

Acquire Hemophilia

Question 424

How is ACQUIRED Hemophilia different than the CONGENITAl Hemophilia?

Answer 424

It is caused by a FACTOR INHIBITOR (NOT the Lupus Inhibitor) which reduces Factor VIII levels (unsuccessful correction after MIXING study and there is MUCOCUTANEOUS bleeding, NOT hemarthroses or bleeding into the muscles

Question 425

How is ACQUIRED Hemophilia treated?

Answer 425

IMMUNOSUPPRESSION (for the long run) to eradicate the Inhibitor and for ACUTE BLEEDING episodes - if low inhibitor titers, can use Factor VIII concentrates, but if the inhibitor concentration is high, Factor VIIa concentrate or Prothrombin Complex are used

Question 426

Liver failure affects clotting factors II, VII, IX and X, the prolongation of which coagulation assays is seen?

Answer 426

PT, aPTT and Clotting Time (low fibrinogen)

Question 427

How does liver failure affect platelets?

Answer 427

Indirectly, by causing SPLENOMEGALY which sequesters the platelets - causing DIC

Question 428

Does an elevated INR in patients with liver disease imply therapeutic auto-anticoagulation?

Answer 428

NO (not a good measure of anti-coagulation)

Question 429

If FFP is needed in patients with liver disease (elevated INR) what else MUST be given?

Answer 429

Vitamin K

Question 430

Abnormal THROMBIN generation with RAPID CONSUMPTION of clotting factors and platelets and ACCELERATED fibrinolysis with SCHISTOCYTES (30% of patients)?

Answer 430

DIC

Question 431

LOW PLATELETS, Prolonged PT, LOW FIBRINOGEN level and ELEVATED D-DIMER?

Answer 431

DIC

Question 432

A malnourished patient (doesn't eat green, leafy vegetables) or a patient with Steatorrhea (fat malabsorption) or a patient who takes chronic systemic ANTIBIOTICS presents with a VERY HIGH INR after only 1-2 days of Warfarin administration, why?

Answer 432

Vitamin K Deficiency (D E A K - fat soluble vitamins)

Question 433

Eclampsia, Retained Birth Products, Fetal Demise, Amniotic Fluid Embolus, Placental Abruption, Infections (Ricketsial, Dengue, Ebola), Crush Injuries, Severe Burns or Brain Injury, Solid Tumors, Acute ProMyelocytic Leukemia (APL), Snake Bites or the Bite of the BROWN RECLUSE spider can all cause what bleeding disorder?

Answer 433

DIC

Question 434

What type of BLEEDING does platelet DEFICIENCY or DYSFUNCTION cause?

Answer 434

MUCO-CUTANEOUS bleeding

Question 435

Platelet count

Answer 435

Thrombocytopenia (underproduction, destruction, splenic sequestration)

Question 436

Symptoms of mucocutaneous bleeding occur below what platelet count?

Question 437

Wet Purpura (petechiae WITHIN the oral mucosa) signifies a risk of what?

Answer 437

SPONTANEOUS Intracranial Hemorrhage (platelets

Question 438

With any suggestion of a cytopenia or cytosis, what two lab tests are essential?

Answer 438

CBC with differential and Peripheral smear

Question 439

If Thrombocytopenia occurs with Anemia and Leukopenia, what could the problem be?

Answer 439

Bon Marrow Failure

Question 440

If Thrombocytopenia is found to have accompanying LEUKOCYTOSIS, what could the problem be?

Answer 440

Acute or Chronic Leukemia

Question 441

What process in a lab can cause false results of thrombocytopenia?

Answer 441

Platelet CLUMPING (pseudothrombocytopenia) caused by inadequate anticoagulation of the sample

Question 442

What do the presence of SPHEROCYTES indicate on a peripheral blood smear?

Answer 442

An Autoimmune Hemolysis

Question 443

An autoimmune process with Ab's against PLATELETS with resulting platelet destruction with increased Bone Marrow platelet production (large, YOUNG platelets) with mild to severe bleeding, usually caused by Drugs or Infection?

Answer 443

Immune Thrombocytopenic Purpura (ITP)

Question 444

What should be done for a patient with diagnosed ITP (Immune Thrombocytopenic Purpura) but without evidence of bleeding and platelet counts ≥30,000/µL?

Answer 444

Counseling on potential symptoms of bleeding and CLOSE observation (repeated CBC every 1-2 weeks)

Question 445

How do you treat a patient with diagnosed ITP (Immune Thrombocytopenic Purpura) with evidence of bleeding and platelet counts

Answer 445

PREDNISONE or dexamethasone + IVIG (if no response to steroids ALONE)

Question 446

What immunosuppressive drugs can be given to patients with refractory ITP (Immune Thrombocytopenic Purpura)?

Answer 446

RITUXIMAB or Mycophenolate Mofetil

Question 447

ß-lactam antibiotics, cephalosporins, sulfa-drugs, vancomycin, quinidine, GPIIbIIIa Inhibitors can all cause this autoimmue bleeding disorder and must be discontinued in a patient who develops it?

Answer 447

ITP (Immune Thrombocytopenic Purpura)

Question 448

What are ROMIPLOSTIM and ELTROMBOPAG?

Answer 448

Platelet production stimulators used in refractory ITP (Immune Thrombocytopenic Purpura)

Question 449

What is the ULTIMATE treatment for a patient with ITP (Immune Thrombocytopenic Purpura) who does not respond to ANY medication or in whom medication toxicities are a concern?

Answer 449

SPLENECTOMY (75% remission)

Question 450

What complication are patients who receive Unfractionated HEPARIN in the setting of CARDIOTHORACIC surgery or after ORTHOPEDIC surgery at risk for 5-10 DAYS after having received HEPARIN?

Answer 450

Heparin-Induced Thrombocytopenia (HIT)

Question 451

5-10 days after having received HEPARIN, a patient's PLATELET count was noted to be 50% LESS than prior to having started HEPARIN and he had developed paradoxical arterial or venous thrombotic events?

Answer 451

Heparin-Induced Thrombocytopenia (HIT)

Question 452

What causes HIT?

Answer 452

An Ab directed against the heparin-platelet complex

Question 453

Hives and angioedema, skin necrosis (as seen with warfarin use), thrombotic episodes even after discontinuation of heparin and thrombocytopenia occurring BEFORE the typically seen 5-10 days after heparin exposure if previously treated with heparin are all atypical presentations of what?

Answer 453

Heparin-Induced Thrombocytopenia (HIT)

Question 454

What MUST be done to treat a patient who developed Heparin-Induced Thrombocytopenia (HIT)?

Answer 454

STOP heparin and START another anticoagulant like LEPIRUDIN (renal clearance) or ARGATROBAN (liver clearance) or danaparoid

Question 455

Is there a way to assess the risk of a patient developing HIT prior to using unfractionated HEPARIN?

Answer 455

YES!! the "4T-score" (0-8 points 6-8 is high probability)

Question 456

IF a patient had been exposed to unfractionated HEPARIN and develops HIT with subsequent unfractionated HEPARIN administration, how soon after the subsequent administration can they develop HIT?

Answer 456

≤1 DAY!!

Question 457

The ABNORMAL ACTIVATION of platelets with DEPOSITION of FIBRIN in the microvasculature and peripheral DESTRUCTION of RBCs and PLATELETS caused by an auto-Ab against a PROTEASE that regulates vWF function allowing vWF to accumulate thereby causing adhesion and activation of platelets?

Answer 457

Thrombotic Thrombocytopenic Purpura (TTP)

Question 458

Patients with bone marrow/solid organ transplants, HIV/AIDS or those taking quinine mitomycin C, cyclosporine or gemcytabine can develop this autoimmune-mediated abnormal activation of platelets by increasing the active form of vWF?

Answer 458

Thrombotic Thrombocytopenic Purpura (TTP)

Question 459

ANY patient with hemolytic anemia with SCHISTOCYTES on peripheral blood smear, LDH and thrombocytopenia should be checked for what?

Answer 459

Thrombotic Thrombocytopenic Purpura (TTP)

Question 460

A patient presenting with FEVER, NEUROLOGIC manifestations (confusion, seizures, sleepiness, coma, stroke) and HEMATURIA with low platelets, peripheral blood smear showing SCHISTOCYTES, anemia and elevated LDH?

Answer 460

Thrombotic Thrombocytopenic Purpura (TTP)

Question 461

What is the treatment of Thrombotic Thrombocytopenic Purpura (TTP)?

Answer 461

PLASMA EXCHANGE + FFP (if neurological symptoms or clinical decline)

Question 462

What happens if PLATELETS are given to a patient with TTP?

Answer 462

FURTHER INCREASED CLOTTING (you're feeding the fire) with resultant thrombosis, stroke, death

Question 463

E.coli or Shigella DIARRHEA with ACUTE RENAL FAILURE with HEMATURIA, FEVER, NO NEUROLOGICAL issues, with anemia and LOW PLATELETS?

Answer 463

Hemolytic Uremic Syndrome (HUS)

Question 464

How do you treat Hemolytic Uremic Syndrome (HUS)?

Answer 464

PLASMA EXCHANGE

Question 465

How do you treat BOTH Thrombotic Thrombocytopenic Purpura (TTP) & Hemolytic Uremic Syndrome (HUS)?

Answer 465

PLASMA EXCHANGE

Question 466

No matter if the diagnosis may be something else (HELLP, malignant HTN, pre-eclampsia, antiphospholipid syndrome or scleroderma renal crisis) IF it at all looks like TTP, what should be done WITHOUT DELAY?

Answer 466

PLASMA EXCHANGE

Question 467

What is "Primary Hemostasis"?

Answer 467

Clotting by way of platelets and platelet adhesion (vWF)

Question 468

What are the TWO (2) screening tests that assess for disorders of clotting due to platelet dysfunction, von Willebrand Disease or thrombocytopenia and anemia (qualitiative)?

Answer 468

Bleeding TIME (not used much) and Platelet Function Analyzer - 100 (newer test)

Question 469

If a patient presents with a personal or a family history of excessive mucocutaneous or post-surgical bleeding problems they most likely have a dysfunction of what?

Answer 469

Platelets

Question 470

When is the Platelet Function Analyzer - 100 best used when assessing for platelet dysfunction?

Answer 470

When it is SEVERE (mild cases may be missed)

Question 471

What effect does uremia have on platelets?

Answer 471

It causes dysfunction (can be treated with or DESMOPRESSIN or dialysis)

Question 472

What happens to platelets in myeloproliferative or myelodysplastic syndromes?

Answer 472

Platelets can be dysfunctional (pt can be treated with platelet transfusions)

Question 473

What effect do NSAIDS have on platelet function?

Answer 473

A reversible one (unlike Aspirin)

Question 474

Blood stasis, Compromised vessel-wall integrity and hypercoagulability all result in what?

Answer 474

Thrombosis

Question 475

Anti-thrombin deficiency, Protein C and Protein S deficiencies, Factor V Leiden (a mutation) and Prothrombin Gene Mutation are inherited what?

Answer 475

Hypercoagulable "Thrombophilic States" (clot-forming) and commonly identified in patients with Venous Thromboembolism (VTE)

Question 476

What does Factor V Leiden (a mutation) do and why is its deficiency important?

Answer 476

It disrupts the sites of cleavage that ACTIVATED PROTEIN C is supposed to cleave on the ACTIVATED Factor V (and ultimately Factor VIII) thereby HALTING unnecessary coagulation. It's deficiency allows activated factors V and VIII (Va and VIIIa) to continue to cause clot formation leading to a HYPERCOAGULABILITY state

Question 477

Can Factor V Leiden deficiency cause ARTERIAL thromboembolism?

Answer 477

NO!! (only venous)

Question 478

Patients with Factor V Leiden (a mutation) should be advised to STAY AWAY from what two things?

Answer 478

Prolonged motionlessness during TRAVEL and Oral Contraceptive Pills

Question 479

How do unfractionated HEPARIN and LMWH work?

Answer 479

They accelerate ANTITHROMBIN activity (an endogenous antithrombotic protein that inhibits THROMBIN and Factor Xa)

Question 480

Unprovoked Thromboembolism and RECURRENT Thromboembolism in young patients (15-35) is usually caused by what?

Answer 480

Inherited ANTITHROMBIN Deficiency

Question 481

What is the function of Protein C?

Answer 481

It inactivates activated Factors Va and VIIIa

Question 482

What does Protein C deficiency cause?

Answer 482

Initial and recurrent Venous Thromboembolism

Question 483

What is the function of Protein S?

Answer 483

It is a co-factor of Protein C and together with activated Protein C (APC), inactivate activated Factors Va and VIIIa

Question 484

Hip & Knee arthroplasty, Trauma and Cancer surgery create the highest risk of what post-op?

Answer 484

Venous Thromboembolism

Question 485

How long after major inpatient surgery, is the risk for Venous Thromboembolism high? Ambulatory surgery?

Answer 485

1 YEAR; 3 MONTHS

Question 486

Trauma to which structures confers the highest risk for Venous Thomboembolism?

Answer 486

Lower Extremities and Pelvic FRACTURES, Spinal Cord injuries (immobility), major Venous Repairs

Question 487

Which cancers have the highest prothrombotic states?

Answer 487

Pancreatic and brain

Question 488

How do you treat patients with cancers and Venous Thromboembolism?

Answer 488

Anticoagulation

Question 489

An acquired autoimmune disorder associated with venous or arterial thromboembolism, pregnancy loss, thrombocytopenia, renal insufficiency, vasculitis and cardiac valvular abnormalities sometimes associated with SLE?

Answer 489

Antiphospholipid Ab Syndrome (APS) (Ab against cardiolipin or glycoproteins)

Question 490

How is Antiphospholipid Ab Syndrome detected?

Answer 490

Elevated aPTT or Russell Viper Venom Time

Question 491

What is needed for diagnosis of Antiphospholipid Ab Syndrome besides Ab's detected in laboratory studies?

Answer 491

Clinical criteria (because it is found transiently in up to 5% of ASYMPTOMATIC adults

Question 492

What is the most common clinical manifestation of Antiphospholipid Ab Syndrome?

Answer 492

Venous Thromboembolism

Question 493

How is Antiphospholipid Ab Syndrome treated?

Answer 493

With LMWH or Fondaparinux (a new LMWH) or Warfarin (INR 2-3)

Question 494

Commonly, pts with Antiphospholipid Ab Syndrome will have RECURRENT Venous Thromboembolism even with a therapeutic INR of 2-3, what should be done then?

Answer 494

Increase Warfarin to INR of 3-4 or change to LMWH or Fondaparinux (a new LMWH)

Question 495

In a patient who wants to get pregnant and has Antiphospholipid Ab Syndrome, what is used for prophylaxis?

Answer 495

Unfractionated Heparin or LWMH + Aspirin

Question 496

If in a patient with Antiphospholipid Ab Syndrome, the Ab's themselves cause elevation in aPTT or INR, what can be measured to assure proper anticoagulation levels when treating with WARFARIN?

Answer 496

Factor X levles

Question 497

A young woman has recurrent miscarriages at or beyond the 10th week of pregnancy with normal fetal morphology and has a history of venous thromboebolism and renal insufficiency?

Answer 497

Antiphospholipid Ab Syndrome

Question 498

What should be done if a patient with Antiphospholipid Ab Syndrome stops their anticoagulation or gets an infection or is s/p surgery and presents with DISSEMINATED microvascular thrombosis and multiorgan failure?

Answer 498

Start anticoagulation, HIGH-dose steroids, IVIG and PLASMA EXCHANGE

Question 499

What does the Russell Venom Assay detect?

Answer 499

Antiphospholipid Ab Syndrome due to Lupus anticoagulant

Question 500

What patients should D-dimer testing be done in as a FIRST diagnostic test of choice when evaluating for DVT or PE?

Answer 500

Those at LOW risk for a first DVT

Question 501

What should be done when evaluating for DVT or PE and the D-dimer test is NEGATIVE?

Answer 501

NOTHING MORE, no chance of DVT or PE

Question 502

What should be done when evaluating for DVT or PE and the D-dimer test is POSITIVE?

Answer 502

Duplex US for DVT or CHEST CT with contrast for PE

Question 503

If a patient is HIGH-RISK for possible DVT or PE, what should be the FIRST diagnostic test of choice?

Answer 503

Duplex US for DVT or CHEST CT with contrast for PE

Question 504

What are LEPIRUDIN, Argatroban and BIVALIRUDIN used for?

Answer 504

Treatment of HIT

Question 505

What are the main anticoagulants used to treat DVT and PE?

Answer 505

Unfractionated Heparin, LMWH, Fondaparinux and Warfarin

Question 506

What are dabigatran and rivaroxaban?

Answer 506

Two agents that can be used INSTEAD of warfarin for the treatment of NON-VALVULAR a-fib and for venous thromboembolism

Question 507

This medication binds to antithrombin and accelerates the inhibition of activated Factor Xa and thrombin and is checked with what laboratory assay?

Answer 507

Unfractionated Heparin, checked with aPTT (weight-based dosing, half life of 60 minutes

Question 508

What is the half-life of LMWH and what patients should it be used CAUTIOUSLY in?

Answer 508

3-6 HOURS, use cautiously in patients with renal insufficiency (renally eliminated)

Question 509

What is the OPTIMAL target aPTT for use with unfractionated Heparin?

Answer 509

51-70 sec (aPTT), if above of below this range, adjust as needed

Question 510

What does Fondaparinux do?

Answer 510

Exclusively catalyzes the antithrombin inhibition of Factor Xa, not thrombin

Question 511

What is the half life of Fondaparinux and whom should it be used cautiously in?

Answer 511

17-21 hours, use cautiously in patients with renal insufficiency (renally eliminated)

Question 512

This medication inhibits the hepatic enzyme that recycles Vitamin K which is ESSENTIAL in the production of Factors II, VII, IX, X and Proteins C & S?

Answer 512

Warfarin

Question 513

What makes Warfarin susceptible to drug interactions requiring caution and dietary modification?

Answer 513

Because of its metabolism via the Cytochrome P-450 system

Question 514

What should be advised to patients taking warfarin in regards to vitamin K-containing foods?

Answer 514

To eat them CONSISTENTLY rather than avoid them (easier to monitor and predict a correct INR

Question 515

When can parenteral therapy be discontinued with unfractionated Heparin as warfarin is given concomitantly?

Answer 515

When the INR is 2 for 24 hours

Question 516

When should the INR be checked in a patient who was recently started on Warfarin?

Answer 516

Weekly, if stable then Monthly and if stable (no dose changes) every 12 WEEKS (ok if SINGLE out-of-range INR ± 0.5, continue same dose and re-checkin 1-2 weeks)

Question 517

How should WARFARIN anticoagulation be reversed in a patient with significant hemorrhage?

Answer 517

STOP warfarin, give 10 mg IV Vitamin K over 1 HOUR as well as FFP

Question 518

How long does it take for a WARFARIN dose change to be manifested?

Answer 518

7-10 days

Question 519

NSAIDs, PPIs, Alcohol, Cipro, Fibrates, Erythromycin, Fluconazole, INH, Metronidazole, Propranolol, Tamoxifen, TMP-SMX all do what to the INR of a patient on WARFARIN?

Answer 519

INCREASE it

Question 520

Carbamazepine, Cholestyramine, Mercaptopurine, Mesalamine, Rifampin and Rifabutin all do what to the INR f a patient taking WARFARIN?

Answer 520

DECREASE it

Question 521

For INPATIENTS, what should be given as venous thromboembolism prophylaxis?

Answer 521

Unfractionated Heparin or LMWH

Question 522

In SURGICAL INPATIENTS ( those s/o STROKE and patients with CANCER, what is the best Venous Thromboembolism prophylaxis agent?

Answer 522

LMWH

Question 523

Besides LMWH, what two other agents are also appropriate s/p ORTHOPEDIC surgery for venous thromboembolism prophylaxis?

Answer 523

WARFARIN (INR 2-3) and Fondaparinux

Question 524

Regardless of surgery type and for all inpatients who have renal impairment with CrCl ≤30 mL/min, what is the recommended prophylaxis for venous thromboembolism?

Answer 524

Unfractionated Heparin

Question 525

When is MECHANICAL prophylaxis for venous thromboembolism preferred over pharmacologic prophylaxis?

Answer 525

ONLY when pharmacologic prophylaxis is CONTRAINDICATED

Question 526

How long should patients undergoing elective HIP arthroplasty, HIP fracture surgery, KNEE arthroplasty and ABDOMINAL/PELVIC CANCER surgery be given venous thromboembolism prophylaxis for?

Answer 526

30-35 days

Question 527

How is unfractionated Heparin and LMWH reversed emergently?

Answer 527

Protamine sulfate

Question 528

How is FONDAPARINUX reversed emergently?

Answer 528

Factor VIIa (activated Factor VII)

Question 529

How is WARFARIN reversed emergently?

Answer 529

Vitamin K 10 mg IV over 1 HOUR + (Prothrombin Complex Concentrate (PCC) or Factor VIIa)

Question 530

How are DIRECT THROMBIN INHIBITORS ( DABIGATRAN, LEPIRUDIN, BIVALIRUDIN) reversed emergently?

Answer 530

Prothrombin Complex Concentrate (PCC) or Hemofiltration/Hemodialysis

Question 531

What is considered CONTRAINDICATION to Pharmacologic prophylaxis for venous thromboembolism?

Answer 531

ACTIVE/HIGH-risk for bleeding, Coagulopathy (abnormal aPTT or PT NOT due to Lupus anticoagulant) or Thrombocytopenia

Question 532

What are the conditions for which HIGH-risk patients receiving WARFARIN require BRIDGING with IV unfractionated Heparin or FULL DOSE LMWH?

Answer 532

Patients with MECHANICAL Heart Valve, a-fib or Venous Thromboembolism who are HIGH-risk (if low risk, no bridging needed

Question 533

If a patient has an uncomplicated DVT, could they be treated as an outpatient?

Answer 533

YES

Question 534

If a patient has a stable PE, can they be treated as an outpatient?

Answer 534

NO!

Question 535

Best choices for treatment of ACUTE Venous Thromboembolism?

Answer 535

LMWH (reversed with protamine) or Fondaparinux (reversed with Factor VIIa)

Question 536

Best choice for treatment of CHRONIC Venous Thromboembolism?

Answer 536

WARFARIN

Question 537

How long MUST parenteral anticoagulation for Venous Thromboembolism overlap with Warfarin until it can be stopped and what INR must be achieved?

Answer 537

5 DAYS, INR of 2.0

Question 538

What is the PREFERRED anticoagulant for Venous Thromboembolism prophylaxis in patients with advanced cancer?

Answer 538

LMWH

Question 539

DVT's and PE's that are IDIOPATHIC or associated with SURGERY or TRAUMA should be treated for how long?

Answer 539

At LEAST 3 MONTHS

Question 540

How long should patients with CANCER who developed Venous Thromboembolism or PE be treated with prophylactic anticoagulation?

Answer 540

For the duration of the cancer/treatment

Question 541

How long should patients with RECURRENT Venous Thromboembolism be treated for with prophylactic anticoagulation?

Answer 541

INDEFINITELY

Question 542

How is SUPERFICIAL THROMBOPHLEBITIS treated?

Answer 542

Compression Stockings and NSAIDS (can be treated with anticoagulation if needed)

Question 543

What is considered physiologic anemia in pregnancy and how long does it last?

Answer 543

Normochromic and Normocytic anemia with a Hb >10.5 g/dL and lasts 8 weeks until a few weeks before delivery

Question 544

What is the most common PATHOLOGIC etiology of anemia in PREGNANT women?

Answer 544

IRON deficiency (requirement exceeds intake)

Question 545

How is IRON deficiency anemia treated in pregnancy?

Answer 545

Oral IRON supplementation

Question 546

When would a patient whether pregnant or not, require parenteral iron supplementation (DEXTRAN) and what is the risk?

Answer 546

If they had malabsorption; risk of anaphylaxis (also pregnancy Class C)

Question 547

Do women with sickle cell trait Hb(AS) have increased risk of obstetric complications or anemia?

Answer 547

NO! [but all others do - Hb(SS) - the worst, Hb(Sߺ), Hb(Sß*), Hb(SC)]

Question 548

What opioid is NOT used in pregnant women when treating Sickle Cell Pain Crises because it can lower the seizure threshold?

Answer 548

Meperidine

Question 549

What Sickle Cell medication MUST be stopped 3 MONTHS before conception?

Answer 549

HYDROXYUREA

Question 550

When should transfusions be given to pregnant women who have Sickle Cell Disease?

Answer 550

If SEVERELY anemic with symptoms of HF

Question 551

Is thrombocytopenia physiologic in pregnancy?

Answer 551

YES, but not

Question 552

A thrombocytopenia that DEVELOPS in the FIRST TWO (2) TRIMESTERS of pregnancy OR a platelet count

Answer 552

Immune Thrombocytopenic Purpura (ITP)

Question 553

How is Immune Thrombocytopenic Purpura (ITP) treated in pregnancy?

Answer 553

Corticosteroids and IVIG

Question 554

HTN, peripheral edema, proteinuria, in the THIRD(3) trimester of pregnancy?

Answer 554

PREECLAMPSIA

Question 555

Schistocytes and helmet cells with thrombocytopenia as well as KIDNEY dysfunction, FEVER and NEUROLOGICAL findings developing in the FIRST (1) or SECOND (2) trimesters?

Answer 555

TTP-HUS

Question 556

RUQ pain, elevated liver enzymes, hemolysis (elevated LDH, schistocytes) with NORMAL PT and aPTT (unlike in AFLP) and low platelets in pregnancy?

Answer 556

HELLP Syndrome (liver biopsy - necrosis, fibrosis, steatosis, hemorrhage)

Question 557

Hypoglycemia, significantly PROLONGED PT and aPTT, elevated LFT's (AST, ALT, T.bili, Alk. Phos.) N/V, Jaundice, Fever in pregnancy?

Answer 557

Acute Fatty Liver of Pregnancy

Question 558

How is TTP-HUS treated in pregnancy?

Answer 558

PLASMA EXCHANGE

Question 559

How are Acute Fatty Liver of Pregnancy, HELLP and PREECLAMPSIA treated?

Answer 559

DELIVERY

Question 560

What is the risk of Venous Thromboembolism in pregnancy?

Answer 560

HIGH (4-5x/normal) with physiologic decreased Protein S activity

Question 561

What congenital THROMBOPHILIC syndrome makes the risk of Venous Thromboembolism VERY HIGH in pregnancy?

Answer 561

Factor V Leiden

Question 562

SLE, Heart Disease, Sickle Cell Disease, Obesity, Anemia, Age ≥35, DM, HTN, smoking and being BLACK raises a pregnant woman's risk of what?

Answer 562

Venous Thromboembolism

Question 563

WHEN during PREGNANCY is the risk for Venous Thromboembolism the HIGHEST?

Answer 563

From the THIRD (3) trimester to 12 WEEKS postpartum

Question 564

ALL pregnant women who have HAD a prior Venous Thromboembolism, should be PRESCRIBED anticoagulation postpartum for HOW LONG?

Answer 564

6 WEEKS If very high risk, it is prescribed BEFORE and AFTER DELIVERY

Question 565

What is the preferred anticoagulant for Venous Thromboembolism Prophylaxis in PREGNANT women?

Answer 565

ENOXAPARIN (LMWH)

Question 566

What are the preferred testing modalities for PREGNANT women who are suspected of having a PE if their LE US was negative?

Answer 566

Ventilation Perfusion Scan or CT angiography

Question 567

If a Venous Thromboembolism occurs close to time of delivery, what is a preferred option for treatment of the mother until after delivery?

Answer 567

Vena Cava Filter

Question 568

Is warfarin a lactation-safe medication?

Answer 568

YES!!

Question 569

If a pregnant woman develops a Venous Thromboembolism DURING pregnancy, how long should she be treated for?

Answer 569

AT LEAST 6 MONTHS and for AT LEAST 6 WEEKS after DELIVERY

Question 570

Which two (2) cancers involve sampling of the SENTINEL Lymph Node (the FIRST node involved in the drainage of these cancers)?

Answer 570

Breast and Melanoma

Question 571

What is the PREFERRED PRIMARY treatment modality for MALIGNANCY?

Answer 571

Surgical Resection

Question 572

What is the utility of radiation therapy prior to resection?

Answer 572

Minimizing risk of local recurrence

Question 573

What is the role of ADJUVANT Chemotherapy and when is it used?

Answer 573

It is used if there is NO demonstrable evidence of remaining cancer after surgical resection, as it increases the statistical chances of CURE for many cancers by DECREASING the risk of any MICROMETASTASES

Question 574

What TEST determines a woman's chances of BENEFITTING from ADJUVANT Chemotherapy for BREAST Cancer?

Answer 574

ONCOtype DX

Question 575

What does the presence of the ESTROGEN receptor confer about the prognosis of a patient with breast cancer?

Answer 575

Better prognosis and a more responsive cancer to therapy

Question 576

What does the presence of the HER2/neu PROTEIN confer about the prognosis of a patient with breast cancer?

Answer 576

Poor Prognosis (aggressive tumor type)

Question 577

What newly-developed CHEMOTHERAPEUTIC drug GREATLY increased the CURABILITY of EARLY HER2/neu positive breast cancers?

Answer 577

TRASTUZUMAB

Question 578

In a small percentage of LUNG adenocarcinoma typically seen in WOMEN who never smoked, as well as in colon, pancreatic, head and neck cancers, GIST and CML, with a particular EPIDERMAL GROWTH FACTOR RECEPTOR (EGFR), this CHEMOTHERAPEUTIC drug is associated with an 80% response rate?

Answer 578

ERLOTINIB (can cause a pruritic or painful acne-like eruption on the face, scalp and trunk treated with topical tetracycline)

Question 579

Kidney, colon, lung, brain, breast and ovarian cancers have a VASCULAR ENDOTHELIAL GROWTH FACTOR which can be inhibited by a ned CHEMOTHERAEUTIC drug called?

Answer 579

BEVACZUMAB (most common adverse effect - HTN and RASH - "Hand-Foot Syndrome" treated with emolients)

Question 580

What type of breast cancer CHEMOTHERAPEUTIC drug are is ANASTROZOLE given ONLY to post-menopausal women that can cause hot flushes, joint pains and osteoporosis?

Answer 580

Aromatase Inhibitor (anti-estrogens)

Question 581

Nulliparity, first childbirth after age of 30, early menarche, late menopause, older age, obesity and alcohol use place a woman at higher risk of what type of cancers?

Answer 581

Breast and Ovarian

Question 582

Treatment of what cancer places a woman at higher risk (25%) for developing breast and thyroid cancer?

Answer 582

Hodgkin lymphoma with mantle RADIATION therapy

Question 583

BRCA1 and BRCA2 gene mutations are associated with what cancers?

Answer 583

Breast and Ovarian

Question 584

What screening should patients who received Mantle Radiation for Hodgkin Lymphoma or have the BRCA1/BRCA2 gene mutations undergo and how often?

Answer 584

YEARLY mammography AND breast MRI

Question 585

What reduces the BREAST cancer risk by 90% and OVARIAN cancer by 95% in women who are positive for the BRCA1/BRCA2 gene mutations?

Answer 585

Prophylactic BILATERAL MASTECTOMY and BILATERAL SALPINGO-OOPHERECTOMY

Question 586

This CHEMOTHERAPEUTIC agent, when used prophylactically in high-risk women for 5 years, reduces the incidence of HORMONE-RECEPTOR positive invasive BREAST cancer by 50%?

Answer 586

TAMOXIFEN

Question 587

How is Ductal Carcinoma IN SITU (DCIS) - usually identified through mammography or palpable lesion treated?

Answer 587

Lumpectomy + Radiation Therapy vs. Mastectomy AND TAMOXIFEN for either for 5 years (if HORMONE-positive)

Question 588

What is the preferred treatment for EARLY INVASIVE Breast Cancer?

Answer 588

MASTECTOMY + Sentinel LN biopsy vs Lumpectomy + Sentinel LN biopsy + Whole-Breast RADIATION Therapy

Question 589

For which breast tumors is LUMPECTOMY (breast-conserving therapy) not practical?

Answer 589

Those ≥5 cm, those that involve the nipple/areola, or multiple tumors, pts with SCLERODERMA, SLE or prior chest radiation therapy

Question 590

When is axillary LN dissection done for INVASIVE Breast Cancer?

Answer 590

If the SENTINEL LN is POSITIVE

Question 591

What if mastectomy is done for INVASIVE Breast Cancer and margins of resection are found to be positive, what should be done?

Answer 591

RADIATION Therapy

Question 592

Stage I INVASIVE Breast Cancer?

Answer 592

Tumor

Question 593

Stages II-III INVASIVE Breast Cancer?

Answer 593

Tumor 2-5 cm AND postitive LNs, involve chest wall/skin

Question 594

Stage IV INVASIVE Breast Cancer?

Answer 594

DISTANT Metastases

Question 595

How are STAGE I-III INVASIVE Breast Cancers treated?

Answer 595

BOTH Adjuvant Endocrine AND Adjuvant Chemotherapy DEPENDING ON RECEPTOR EXPRESSION!!!!!

Question 596

Why is the use of AROMATASE inhibitors (ANASTROZOLE) useful in POST-Menopausal women only?

Answer 596

Because their estrogen/progesterone production is no longer ovarian but rather produced by fat and muscle

Question 597

Can AROMATASE inhibitors be used in PRE-Menopausal women to treat hormone-receptor POSITIVE INVASIVE Breast Cancer?

Answer 597

NO (no effect on estrogen/progesterone produced by ovaries)

Question 598

What ADJUVANT Endocrine CHEMOTHERAPEUTIC agent is used in PRE-Menopausal women and ALL MEN?

Answer 598

TAMOXIFEN (ESTROGEN Receptor ANTAGONIST)

Question 599

What ELSE is used as ADJUVANT Endocrine CHEMOTHERAPY in POST-Menopausal women besides the AROMATASE inhibitors?

Answer 599

TAMOXIFEN is used as well

Question 600

How LONG are AROMATASE inhibitors and TAMOXIFEN used for in POST-Menopausal women with INVASIVE Breast Cancer?

Answer 600

5 years each, one after the other starting with AROMATASE inhibitor then TAMOXIFEN

Question 601

Hot flushes, increased thromboembolic events, endometrial cancer and cataracts are all adverse effects of this CHEMOTHERAPEUTIC drug used to treat breast cancer?

Answer 601

TAMOXIFEN

Question 602

What is the only significant adverse effect of AROMATASE inhibitors?

Answer 602

Osteoporosis (needs an added bisphosphonate)

Question 603

What should ALL patients with HER2/neu protein over-expression POSITIVE INVASIVE Breast Cancer be treated with as PART of their ADJUVANT Chemotherapy regimen?

Answer 603

TRASTUZUMAB + 2 others (cyclophosphamide, anthracyclines (rubicins), methotrexate, 5-FU and a taxane (paclitaxel)

Question 604

Bone marrow suppression, Neurotoxicity, Neutropenic fever (due to BM suppression), amenorrhea are all adverse effects of this class of CHEMOTHERAPEUTIC drugs?

Answer 604

Taxanes (pacliTAXEL, doceTAXEL)

Question 605

What is DIFFERENT about the CARDIOTOXICITY (Systolic ventriculat dysfunction, HF) that TRASTUZUMAB causes than the ANTHRACYCLINES (-"rubicin")?

Answer 605

Cardiotoxicity imparted by TRASTUZUMAB is REVERSIBLE

Question 606

Patients with EITHER "LOCALLY-ADVANCED" (chest wall/skin involvement ± multiple LNs) Breast Cancer AND INFLAMMATORY Breast Cancer (erythema, skin thickening, peau d'orange) are treated in a unique way, how?

Answer 606

They are given PRE-SURGICAL CHEMOTHERAPY

Question 607

How is a patient with the INFLAMMATORY sub-type of INVASIVE Breast Cancer (erythema, skin thickening, peau d'orange) treated?

Answer 607

MULTI-Modality - Systemic CHEMOTHERAPY + MASTECTOMY + Radiation Therapy

Question 608

How long and how frequently should early-stage breast cancer survivors be seen in follow-up?

Answer 608

Every 6 months for 5 years, then yearly with yearly mammography and breast self-exams

Question 609

As systemic HORMONE therapy is CONTRAINDICATED in ANY BREAST/OVARIAN cancer survivor, is it ok to use estrogen-based vaginal creams?

Answer 609

YES

Question 610

Which is the ONLY anti-depressant that is RECOMMENDED for use for vasomotor symptoms (HOT FLUSHES) in a woman using TAMOXIFEN?

Answer 610

VENLAFAXINE (SNRI) - metabolized via CYP450, not the CYP2D6 needed for tamoxifen

Question 611

Can early-breast cancer survivors use oral contraceptive pills?

Answer 611

NO! (these are hormone-based)

Question 612

What adverse effect can chemotherapy used to treat breast cancer cause as related to fertility?

Answer 612

Amenorrhea and early menopause

Question 613

What treatments are not performed on metastatic breast cancer?

Answer 613

Surgery or Radiation therapy (no survival benefit) - median survival is 2 years (done ONLY for palliation or spinal compression symptoms)

Question 614

What must be done to new/recurrent breast cancer lesions?

Answer 614

BIOPSY (new/recurrent lesions may have different receptors)

Question 615

How should patients with Breast Cancer metastatic to the bone be treated?

Answer 615

Adding bisphosphonates (pamidronate/zoledronic acid)

Question 616

What is required for ALL patients, regardless of the reason for treatment, PRIOR to initiating bisphophonate therapy?

Answer 616

Dental evaluation BEFORE and DURING treatment to avoid osteonecrosis of the jaw

Question 617

Usually diagnosed in women >50 (post-menopausal), the highest risk for ovarian cancer is what?

Answer 617

BRCA1/BRCA2 gene mutation (can see younger patients) as well as women with HNPCC

Question 618

What factors increase the risk for ovarian cancer besides the BRCA1/BRCA2 gene mutation?

Answer 618

Obesity, Nulliparity, Late Menopause

Question 619

What medication class reduces the incidence of Ovarian Cancer by 50% however NOT TO BE USED in patients with BRCA1/BRCA2 because of the increased RISK of BREAST CANCER!!!?

Answer 619

Oral Contraceptive Pills (protective effect lasts up to 20 years after discontinuation)

Question 620

What should patients with BRCA1/BRCA2 be advised to do after childbearing is complete AND BEFORE age 40?

Answer 620

BILATERAL Mastectomy and Salpingo-Oopherectomy (reduces risk of breast/ovarian cancer by 90-95%)

Question 621

How should patients who are positive for the BRCA1/BRCA2 gene mutations be managed if they REFUSE surgery (b/l mastectomy and salpingo-oopherectomy) prior to age 40?

Answer 621

INTENSIVE surveillance either at the age of 35, OR 10-YEARS younger than the youngest family member to have developed cancer with pelvic examinations, CA-125 and pelvic US every 6 months.

Question 622

The presence of pelvic/adnexal ascites or mass in a post-meopausal woman is suspicious for what?

Answer 622

OVARIAN cancer (do pelvic US - septated cyst, solid mass, ascites)

Question 623

What should be done if a pelvic US detects the possibility of an ovarian cancer (septated cyst, ascites, solid mass)

Answer 623

CT scan of Abdomen/Pelvis (followed by CT/US-guided biopsy or ascitic fluid sampling)

Question 624

CA-125 ≥65 units/mL?

Answer 624

NON-specific marker, however, found in more than 80% of patients with Ovarian Cancer

Question 625

What is needed for diagnosis, staging and treatment of OVARIAN Cancer?

Answer 625

SURGERY (tumor debulking - total hysterectomy, appendectomy, omentectomy and LN dissection)

Question 626

Is chemotherapy used for Early Stage I Ovarian Cancer?

Answer 626

NO (no benefit) only for those wit hHIGH-risk early stage disease and advanced disease

Question 627

What is the difference between neoadjuvant and adjuvant chemotherapy?

Answer 627

NEOADJUVANT is given BEFORE SURGERY (or main treatment) and ADJUVANT is given AFTER (to prevent relapse)

Question 628

What is the ADJUVANT chemotherapy regimen used in ADVANCED (stages II, III and IV) Ovarian Cancer?

Answer 628

A Taxane (pacliTAXEL) + cisPLATIN or carboPLATIN

Question 629

What should be done for RECURRENT Ovarian Cancer?

Answer 629

SURGICAL resection (if solitary tumor with limited spread) as well as PALLIATIVE chemotherapy

Question 630

How are Ovarian Cancer patients monitored AFTER initial therapy?

Answer 630

By FREQUENT measurement of CA-125 levels (every 2-6 months)

Question 631

Does TOTAL Parenteral Nutrition have a survival benefit for CANCER?

Answer 631

NO

Question 632

Can the use of hematopoietic growth factors (epoetin or darbopoetin) improve the quality of life of Ovarian Cancer patients undergoing CHEMOTHERAPY and decrease complication rates due to inadequate blood counts?

Answer 632

YES!

Question 633

What is the most important RISK factor for development of CERVICAL cancer (squamous/adeno)?

Answer 633

HPV

Question 634

Early-onset sexual activity, multiple sexual partners, STIs, smoking, MULTIPARITY, prolonged use of Oral Contraceptive Agents and Immunosuppression are all risk factors for what cancer?

Answer 634

CERVICAL cancer (squamous cell carcinoma 80%, adenocarcinoma 20%)

Question 635

A woman with vaginal bleeding BETWEEN menstrual periods, after MENOPAUSE, POST-COITAL vaginal bleeding and abnormal vaginal discharge are all suspicious for what?

Answer 635

CERVICAL cancer

Question 636

What is Stage I Cervical Cancer?

Answer 636

LIMITED to UTERUS

Question 637

When Cervical Cancer involves the pelvic side wall or LOWER 1/3rd of vagina, what stage is it?

Answer 637

Stage III

Question 638

Extension of Cervical Cancer BEYOND the uterus but NOT involving the LOWER 1/3rd of the vagina (can involve upper 2/3rds) or the pelvic side wall is what stage?

Answer 638

Stage II

Question 639

In what CERVICAL Cancer patients is OBSERVATION alone acceptable if not surgical candidates or wish to maintain fertility?

Answer 639

Stage IA and ONLY after an EXCISIONAL CONE biopsy

Question 640

How are Stage IB, IC and II Cervical Cancers treated?

Answer 640

Radiation therapy OR Radical HYSTERECTOMY ± CISPLATIN chemotherapy

Question 641

If HIGH-Risk factors are identified at time of surgery for CERVICAL Cancer (large tumor, deep tissue invasion, LN involvement or POSITIVE surgical margins) what is done for treatment?

Answer 641

Post-OP RADIATION + CHEMOTHERAPY

Question 642

How are patients monitored after CERVICAL Cancer treatment?

Answer 642

Pelvic Exams and PAP smears every 3-6 months for 2 years then every 6 months for 3 more years, then yearly

Question 643

What is done for treatment of RECURRENT CERVICAL Cancer?

Answer 643

Salvage RADIATION and CHEMOTHERAPY

Question 644

What is considered "HIGH-RISK" for colorectal cancer warranting colorectal cancer screening with COLONOSCOPY at the age of 40, OR 10-YEARS earlier than the youngest family member diagnosed with colon cancer?

Answer 644

Family History of COLON CANCER

Question 645

What is the PRE-OPERATIVE STAGING for colorectal cancer comprised of?

Answer 645

COLONOSCOPY + CT w/Contrast of CHEST/ABD/PELVIS

Question 646

What is Stage I (T1, T2) colon cancer?

Answer 646

Does NOT involve FULL thickness of colon wall, NO LN's

Question 647

What is Stage II (T3, T4) colon cancer?

Answer 647

Involves FULL thickness of colon wall, NO LN's

Question 648

What is the difference between Stages I & II Colon Cancer and Stages II & IV?

Answer 648

Stages II and IV INVOLVE LN's

Question 649

Which COLON Cancer patients REQUIRE ADJUVANT CHEMOTHERAPY after surgical resection?

Answer 649

ALL who have Stage III disease (ANY LN involvement)

Question 650

What is the BEST ADJUVANT CHEMOTHERAPEUTIC treatment for patients with HIGH-RISK Stage II (T4,

Answer 650

FOLFOX (5-FU + folinic acid (leucovorin) + oxaliPLATIN) for 6 MONTHS

Question 651

In patients with RECTAL cancer, with Stage II-IV (full thickness OR LN involvement), what should be done?

Answer 651

COMBINED CHEMOTHERAPY (FOLFOX) + RADIATION BEFORE Surgery with CHEMOTHERAPY AFTER Surgery

Question 652

As metastatic colon cancer is typically INCURABLE and requires palliative CHEMOTHERAPY/Surgery, what patients with metastatic colorectal cancer can have potential CURATIVE treatment?

Answer 652

ONLY those with metastatic disease confined to a SINGLE organ (liver or lung)

Question 653

Why is K-ras genotyping done in patients with Stage IV Colon Cancer?

Answer 653

Because in those patients whom do NOT have mutations of this gene, other chemotherapeutic agents can also be used

Question 654

What is the ORAL version of 5-FU (part of the FOLFOX chemotherapy regimen used for colorectal cancer treatment)?

Answer 654

CAPECITABINE

Question 655

What does the development of a ACNE-like rash in patients being treated with MONOCLONAL Ab's to Epidermal Growth Factor Receptors (EGFR) - CHEMOTHERAPEUTIC agents mean?

Answer 655

GOOD Prognosis, GOOD anti-tumor activity

Question 656

Should MONOCLONAL Ab's against Vascular Endothelial Growth Factors (VEGF) and Epidermal Growth Factor Receptors (EGFR) be used together?

Answer 656

NO, DECREASED Survival

Question 657

What should the post-OP monitoring and surveillance be for a patient treated for COLON Cancer?

Answer 657

CEA levels monitored every 3-6 months for the first 3 years and then every 6 months for the next 2 years with CT w/contrast of the CHEST/ABD/PELVIS yearly for the first 3 years and COLONOSCOPY at Year 1, 3 and every 5 Years thereafter

Question 658

What GI cancer are HPV and HIV most commonly involved in?

Answer 658

ANAL Cancer

Question 659

How is ANAL Cancer treated INITIALLY?

Answer 659

COMBINTION Radiation + Chemotherapy (Mitomycin + 5-FU)

Question 660

What gene mutation has been linked to increased PANCREATIC Cancer risk?

Answer 660

BRCA2

Question 661

Is a biopsy required for a pancreatic cancer if it is isolated and resectable after a CT w/contrast is done of the CHEST/ABDOMEN?

Answer 661

NO

Question 662

What is done for PANCREATIC Cancers that involve the Superior Mesenteric Artery or Celiac Trunk?

Answer 662

COMBINED RADIATION therapy and CHEMOTHERAPY prior to SURGERY

Question 663

Is Chemotherapy used AFTER PANCREATIC Cancer resection?

Answer 663

NO

Question 664

What is the standard CHEMOTHERAPY used in patients with METASTATIC PANCREATIC Cancer?

Answer 664

GEMCITABINE

Question 665

For EXTREMELY FIT PANCREATIC Cancer patients with EXCELLENT performance status, what is a BETTER (survival time) CHEMOTHERAPEUTIC regimen than Gemcitabine however with significantly greater toxicity?

Answer 665

FOLFIRINOX (5-FU + leucovorin + irinotecan + oxaliPLATIN)

Question 666

What is the most common ESOPHAGEAL Cancer type?

Answer 666

Adenocarcinoma (GERD and obesity-associated)

Question 667

How are ESOPHAGEAL ADENOCARCINOMA Stages I-III treated?

Answer 667

PRE-OP CHEMOTHERAPY + RADIATION, then SURGERY

Question 668

How are GASTRIC Adenocarcinomas treated?

Answer 668

PRE & POST-OP CHEMOTHERAPY (5-FU + cisPLATIN + epiRUBICIN)

Question 669

If a patient with GASTRIC Adenocarcinoma did NOT undergo PRE-OP CHEMOTHERAPY, what should be done POST-OP for them?

Answer 669

CHEMOTHERAPY (5-FU + leucovorin) AND RADIATION Therapy

Question 670

What should ALWAYS be part of a CHEMOTHERAPEUTIC treatment regimen when HER2/neu is involved?

Answer 670

TRASTUZUMAB

Question 671

What are Carcinoid tumors?

Answer 671

Neuroendoctine Tumors (NETs) derived from the aero-digestive tract

Question 672

Of the 25% of Neuroendocrine Tumors (NETs) that secrete hormones, what hormones are secreted from Carcinoids?

Answer 672

Serotonin (diarrhea and facial flushing)

Question 673

What is the most common site for METASTASES of Neuroendocrine Tumors?

Answer 673

LIVER (carcinoids)

Question 674

What is the most common location for a CARCINOID tumor?

Answer 674

Appendix

Question 675

What is the difference between SURGICAL resection of a 1 cm CARCINOID tumor and one that is ≥2 cm located in the APPENDIX?

Answer 675

1 cm tumors can be resected by APPENDECTOMY alone whereas 2 cm tumors require RIGHT HEMICOLECTOMY (to ensure complete LN removal as well)

Question 676

What are the preferred imaging modalities for CARCINOID Tumors?

Answer 676

Triple-phase contrast CT or Gadolinium MRI

Question 677

How can CARCINOID Tumors that are detected on Indium-111 pentetreotide scan (octerotide scan) be treated if hormonally active?

Answer 677

With OCTREOTIDE (because they have somatostatin receptors)

Question 678

What type of Neuroendocrine Tumors are responsive to CHEMOTHERAPY (Sunitinib and Everolimus)?

Answer 678

Pancreatic NETs

Question 679

Small Cell Lung Cancer (SCLC) and Non-Small Cell Lung Cancer (NSCLC) are related to what risk factor?

Answer 679

SMOKING

Question 680

Patients who develop LUNG Cancer and never smoked or were exposed to 2nd hand smoke develop what type of LUNG Cancer?

Answer 680

Non-Small Cell Lung Cancer (NSCLC)

Question 681

EXPOSURE to asbestos, Radon, Arsenic, Cadmium and Nickel confers the increased risk of what cancer?

Answer 681

LUNG Cancer

Question 682

What is the LEADING cause of CANCER-related DEATH in the US?

Answer 682

LUNG Cancer

Question 683

What should be done for SCREENING in HIGH-RISK individuals (minimum current or past history of 30-pack-year smoking, first-degree relative with lung cancer, significant exposure to radon or asbestos)?

Answer 683

LOW-Dose Spiral CT scan

Question 684

What is the recommendation for INCIDENTALLY-FOUND pulmonary NODULES ≤4 mm?

Answer 684

In HIGH-RISK individuals (smoking, environmental exposures) - REPEAT imaging in 12 MONTHS In LOW-RISK individuals (never smokers, no 1st degree relatives with lung cancer, no significant Radon or Asbestos exposure) - NO follow-up imaging required

Question 685

Cough with hemoptysis, pleural effusion, chest pain and hoarseness (recurrent laryngeal nerve), brachial plexus pain, Horner's syndrome (miosis, ptosis, anhidrosis)?

Answer 685

LUNG Cancer (miosis, ptosis, anhydrosis is associated with a Pancoast tumor in the lung APEX - Horner's syndrome)

Question 686

Weight loss, bone pain, neurologic symptoms are seen in 25% of patients who present with advanced?

Answer 686

LUNG Cancer

Question 687

What LUNG Cancer can present with HYPERCALCEMIA due to bone METS but also due to the PARANEOPLASTIC effects of a parathyroid-related protein?

Answer 687

Non-Small Cell Lung Cancer (NSCLC)

Question 688

Paraneoplastic syndromes such as SIADH (with profound HYPOnatremia) and Eaton-Lambert Myesthenic Syndrome (proximal muscle weakness that IMPROVES with activity (opposite from Myesthenia Gravis) are seen in what type of Cancer?

Answer 688

Small Cell Lung Cancer (SCLC)

Question 689

Clubbing of the digits, Periostitis (bone pain due to inflammation of the periosteum) and Joint pain are seen with this adenocarcinoma?

Answer 689

Non-Small Cell Lung Cancer (NSCLC) - adenocarcinoma, squamous cell carcinoma, large-cell carcinoma

Question 690

Why is Small Cell Lung Cancer NOT resectable?

Answer 690

Because it DISSEMINATES SYSTEMICALLY before diagnosis even if it appears to be confined to one lung

Question 691

Non-Small Cell Lung Cancer (NSCLC) with a solitary tumor, no REGIONAL (peribronchial, hilar or mediastinal) LN's is what stage?

Answer 691

Stage I (3 cm IB)

Question 692

Non-Small Cell Lung Cancer (NSCLC) with REGIONAL (peribronchial, hilar or mediastinal) LN involvement, OR with involvement of PLEURa, CHEST WALL or near the CARINA are what stage?

Answer 692

Stage II

Question 693

When mediastinal LN's are involved with Non-Small Cell Lung Cancer (NSCLC), what is the stage?

Answer 693

Stage III

Question 694

The presence of a malignant PLEURAL EFFUSION or METS makes a NSCLC what stage?

Answer 694

Stage IV

Question 695

What NSCLC stages are RESECTABLE?

Answer 695

I and II

Question 696

What makes NSCLC NOT RESECTABLE?

Answer 696

PRESENCE of MEDIASTINAL LN's

Question 697

Where does LUNG Cancer metastasize to?

Answer 697

Liver, Bone, Brain, Adrenal Glands

Question 698

What further DIAGNOSTIC studies are done AFTER biopsy confirms Non-Small Cell Lung Cancer (NSCLC)?

Answer 698

CT of the CHEST/ABDOMEN + BONE SCAN or PET/CT + BRAIN MRI with Gadolinium

Question 699

What should be done prior to ANY surgery for Non-Small Cell Lung Cancer (NSCLC)?

Answer 699

Assessment of LUNG FUNCTION to determine if post-surgical lung function would be adequate (TLC, FEV,DLCO)

Question 700

30% of ALL Non-Small Cell Lung Cancers (NSCLC) are Stage I or II, what is the treatment?

Answer 700

SURGERY

Question 701

What is the most COMMON Non-Small Cell Lung Cancer (NSCLC) Stage at presentation?

Answer 701

Stage III (presence of mediastinal LN's)

Question 702

What is the treatment for Non-Small Cell Lung Cancer (NSCLC) Stage III?

Answer 702

COMBINED Radiation and Chemotherapy

Question 703

What should be given to ALL Non-Small Cell Lung Cancer (NSCLC) patients after SURGICAL Resection in Stages II & III?

Answer 703

ADJUVANT cisPLATIN-BASED Chemotherapy

Question 704

How is Stage IV Non-Small Cell Lung Cancer (NSCLC) treated?

Answer 704

PALLIATION ONLY with Chemotherapy (2-drug PLATINUM-based + BEVACIZUMAN/CETUXIMAB)

Question 705

What is Radiation Therapy used for in Stage IV Non-Small Cell Lung Cancer (NSCLC)?

Answer 705

Symptom control (SVC syndrome, pneumonitis, brain mets, spinal cord compression, bone mets)

Question 706

How are symptoms of bone pain treated in LUNG Cancer with BONE mets?

Answer 706

Radiation Therapy + Bisphosphonates

Question 707

What SPECIAL subset of Non-Small Cell Lung Cancer (NSCLC) patients does the Epidermal Growth Factor Receptor (EGFR) Tyrosine Kinase Inhibitor ERLOTINIB help?

Answer 707

ONLY those with a EGFR gene MUTATION (Asian women who never smoked)

Question 708

Small Cell Lung Cancer (SCLC) EXCLUSIVELY affects what patients?

Answer 708

SMOKERS

Question 709

What two stages does Small Cell Lung Cancer (SCLC) have?

Answer 709

Limited and Extensive stages

Question 710

What stage is Small Cell Lung Cancer (SCLC) if it involves one HEMITHORAX with HILAR, MEDIASTINAL and IPSILATERAL SUPRACLAVICULAR LN's?

Answer 710

Limited Stage

Question 711

What stage is Small Cell Lung Cancer (SCLC) when it has an IPSILATERAL Malignant PLEURAL EFFUSION and goes beyond one hemithorax?

Answer 711

Extensive Stage

Question 712

What is Small Cell Lung Cancer (SCLC) considered at diagnosis?

Answer 712

SYSTEMIC Disease

Question 713

How is "LIMITED STAGE" Small Cell Lung Cancer (SCLC) treated?

Answer 713

COMBINED RADIATION and CHEMOTHERAPY (PLATIN + ETOPOSIDE)

Question 714

How is "EXTENSIVE STAGE" Small Cell Lung Cancer (SCLC) treated?

Answer 714

CHEMOTHERAPY alone, Radiation is for symptom palliation only (bone pain, spine, brain)

Question 715

When is PROPHYLACTIC BRAIN Radiation Therapy (prevent mets to brain) used in Small Cell Lung Cancer (SCLC)?

Answer 715

When LIMITED Stage SCLC patients complete combined radiation and chemotherapy and are expected to survive

Question 716

What are the two MAJOR risk factors for head and neck cancers?

Answer 716

Alcohol and Tobacco

Question 717

If a patient presents with a head/neck cancer that is NOT alcohol/tobacco related, what are the two other risk factors?

Answer 717

HPV (16) and EBV

Question 718

Referred ear pain, dysphagia, odynophagia in a smoker?

Answer 718

Suspect Head/Neck cancer

Question 719

How do you diagnose Head/Neck cancer?

Answer 719

Visual inspection-->Endoscopy±biopsy-->CT/MRI-->PET if metastatic

Question 720

What is the difference between Stages I & II and Stages III & IV in Head/Neck cancers?

Answer 720

LN involvement

Question 721

What should be done in a SUSPECTED Head/Neck Cancer if a PALPABLE LN FNA is negative?

Answer 721

LN BIOPSY

Question 722

What is the PRIMARY treatment for a Head/Neck cancer?

Answer 722

SUGERY + Radiation therapy

Question 723

Why are cancers of the larynx/hypopharynx usually treated with radiation therapy rather than surgery?

Answer 723

To preserve organ function (voice)

Question 724

How are Stage III & IV Head/Neck cancers treated?

Answer 724

Radiation & Chemotherapy (PLATIN) FIRST, then RADICAL neck dissection surgery (chemotherapy alone if metastatic)

Question 725

Prostatitis and a high fat and low-fiber diet, being black, of older age and having a first-degree family relative affects raises the risk for this cancer?

Answer 725

Prostate Cancer

Question 726

What can be used for PROPHYLAXIS to reduce the incidence of prostate cancer in HIGH-risk patients?

Answer 726

5-alpha-reductase inhibitors (finasteride) - gynecomastia, decreased libido, erectile dysfunction

Question 727

Should men be screened by PSA for prostate cancer?

Answer 727

NO

Question 728

What should be done in a symptomatic patient in which the digital rectal exam is abnormal?

Answer 728

Refer for UROLOGY evaluation (REGARDLESS OF PSA level)

Question 729

A gentleman comes in with symptoms of urinary hesitancy, incomplete bladder emptying with decreased urinary stream and nocturia, what should be done?

Answer 729

Digital Rectal Exam to check for prostate cancer/BPH

Question 730

New onset sexual dysfunction is a concern for what condition in a male patient?

Answer 730

Prostate Cancer (invasion into the neurovascular bundle)

Question 731

Prostate cancer with bone pain/spinal cord compression?

Answer 731

Metastatic disease (treat BONE symptoms with radiation and bisphopshonates)

Question 732

What is the DEFINITIVE diagnostic test for prostate cancer?

Answer 732

MULTIPLE Trans-RECTAL PROSTATE Biopsies from ALL parts of the PROSTATE

Question 733

Does aspirin have to be stopped prior to trans-rectal prostate biopsies?

Answer 733

NO

Question 734

TNM + Gleason Score + PSA =?

Answer 734

Staging required for PROSTATE Cancer

Question 735

Prostate cancer is neither palpable nor visible on imaging, what is the stage?

Answer 735

T1

Question 736

Prostate cancer is confined to the prostate CAPSULE, what is the stage?

Answer 736

T2

Question 737

Prostate cancer invades through the prostate capsule or invades seminal vesicles, what is the stage?

Answer 737

T3

Question 738

Which is the ONLY PROSTATE Cancer stage that involves ANY LN or metastatic disease?

Answer 738

T4

Question 739

Pt presents with a normal PSA level but c/o urologic symptoms and has an abnormal digital rectal examination, what should be done next?

Answer 739

Urology referral for TRANS-Rectal BIOPSIES of the PROSTATE

Question 740

Pt with LOW-risk PROSTATE Cancer with a SHORT-life expectancy should be treated how?

Answer 740

OBSERVATION ONLY

Question 741

Pt with LOW-risk PROSTATE Cancer with a LONG-life expectancy should be treated how?

Answer 741

RADICAL PROSTATECTOMY, Radiation Therapy or ACTIVE SURVEILLANCE (observe until need to treat)

Question 742

Why would a younger patient choose "ACTIVE SURVEILLANCE" for diagnosed PROSTATE Cancer rather than SURGERY or RADIATION now?

Answer 742

In order to avoid the possible TREATMENT-related complications (erectile dysfunction, impotence, urinary incontinence) in the short term

Question 743

Pt with HIGH-risk PROSTATE Cancer (T3-T4, Gleason score 8-10, PSA ≥20) with a SHORT-life expectancy should be treated how?

Answer 743

ANDROGEN DEPRIVATION therapy vs OBSERVATION

Question 744

Pt with HIGH-risk PROSTATE Cancer (T3-T4, Gleason score 8-10, PSA ≥20) with a LONG-life expectancy should be treated how?

Answer 744

ANDROGEN DEPRIVATION Therapy FIRST (8-months), THEN RADIATION Therapy vs. SURGERY (followed by observation vs radiation and androgen deprivation therapy)

Question 745

What should be recommended to a patient with Sage I-II PROSTATE Cancer (confined to CAPSULE) and with a life expectancy >10 years?

Answer 745

RADICAL PROSTATECTOMY OR Radiation Therapy (external beam or brachytherapy)

Question 746

What is an EQUALLY-GOOD alternative to RADICAL PROSTATECTOMY?

Answer 746

RADIATION THERAPY (External Beam or Brachytherapy)

Question 747

What does ANDROGEN DEPRIVATION therapy consist of in the treatment of PROSTATE CANCER?

Answer 747

GnRH antagonists (cetroRELIX)/agonists (leuprolide, boseRELIN) + ANTI-Androgens (flutamide, ketoconazole)

Question 748

Men receiving ANDROGEN DEPRIVATION therapy must be monitored and treated for what?

Answer 748

OSTEOPENIA (bisphophonates)

Question 749

ALMOST ALL PROSTATE Cancer Survivors WILL develop HORMONE-Refractory Cancer and METASTATIC disease and should be treated how?

Answer 749

CHEMOTHERAPY (doceTAXEL + Prednisone)

Question 750

What is the FIRST Autologous Cellular IMMUNOTHERAPEUTIC agent used for the treatment of HORMONE-REFRACTORY PROSTATE Cancer which works by activating the patient's immune system to target prostate cancer cells?

Answer 750

SIPULEUCEL-T

Question 751

How should post-treatment PROSTATE Cancer patients be monitored?

Answer 751

REGULAR Digital Rectal Examinations and PSA levels

Question 752

Which TESTICULAR Cancer type has the best prognosis?

Answer 752

PURE SEMINOMAS

Question 753

What are CHORIOCARCINOMA, EMBRYONAL Carcinoma, YOLK SAC Carcinoma, TERATOMA or MIXED?

Answer 753

NON-SEMINOMAS (Testicular Cancer)

Question 754

Kleinfelter syndrome (tall, slightly feminized physique with breast growth, small testicles, osteoporosis), Cryptorchidism, Family history are all risk factors for what?

Answer 754

TESTICULAR Cancer

Question 755

Pt with testicular mass p/w COUGH, Back Pain, DYSPNEA, neurologic abnormalities and gynecomastia, what is suspected?

Answer 755

TESTICULAR Cancer

Question 756

What should be done for ALL patients with a PALPABLE testicular mass?

Answer 756

Testicular US, Urology evaluation, CXR, CT abd/pelv and if suspicious CXR, do CHEST CT

Question 757

What serological marker diagnoses a NON-SEMINOMA TESTICULAR Cancer?

Answer 757

Alpha-fetoprotein

Question 758

Alpha-fetoprotein, ß-HCG, LDH (rapid cell turnover) are seen in what cancers?

Answer 758

TESTICULAR Cancers

Question 759

TESTICULAR Cancer confined to the testicle is what stage?

Answer 759

Stage I

Question 760

TESTICULAR Cancer with REGIONAL LN's is what stage?

Answer 760

Stage II

Question 761

TESTICULAR Cancer with METS is what stage?

Answer 761

Stage III

Question 762

In what two (2) ways is the TNM staging system DIFFERENT for TESTICULAR Cancer Staging?

Answer 762

Only Stages I-III (there is NO stage IV) and there is an "S" stage for serological markers

Question 763

What should be done for ALL patients with TESTICULAR Cancer of ANY kind?

Answer 763

RADICAL ORCHIECTOMY

Question 764

How is Stage I SEMINOMA (Testicular Cancer) treated?

Answer 764

RADICAL ORCHIECTOMY + ACTIVE SURVEILLANCE vs. RADIATION Therapy to Para-AORTIC LN's or carboPLATIN

Question 765

How are patients with TESTICULAR Cancer RELAPSE treated?

Answer 765

MULTI-agent CHEMOTHERAPY

Question 766

How is Stage II SEMINOMA (Testicular Cancer) treated?

Answer 766

RADICAL ORCHIECTOMY + RADIATION Therapy OR PLATIN Chemotherapy

Question 767

How and HOW LONG is a patient monitored for after treatment for SEMINOMA TESTICULAR Cancer?

Answer 767

CT abd/pelv 4 months POST-OP and Serum Tumor Markers every 2 MONTHS x 1 year then less frequently over the next 10 YEARS

Question 768

How is Stage IA NON-SEMINOMA (Testicular Cancer) treated?

Answer 768

RADICAL ORCHIECTOMY + ACTIVE SURVEILLANCE/RETROPERITONEAL LN Dissection (RPLND)

Question 769

How is Stage IB NON-SEMINOMA (Testicular Cancer) treated?

Answer 769

RADICAL ORCHIECTOMY + RETROPERITONEAL LN Dissection (RPLND) + MULTI-agent CHEMOTHERAPY (PLATIN, ETOPOSIDE, BLEOMYCIN)

Question 770

What histology type are most urinary bladder cancers?

Answer 770

TRANSITIONAL Cell

Question 771

Men >60 yo, smokers OR metal workers, painters, leather workers with GROSS hematuria should be checked for what?

Answer 771

Urinary BLADDER Cancer

Question 772

What MUST be evaluated in a patient with hematuria?

Answer 772

ALL components of the urinary tract

Question 773

What is involved in diagnosing urinary BLADDER cancer?

Answer 773

CT/MRI or Intravenous Pyelogram + Cystoscopy + Urine Specimen

Question 774

What should be done during cystoscopy if a possibly malignant lesion is seen?

Answer 774

COMPLETE Transurethral Resection of the Bladder Tumor (TURBT) with biopsies of normal tissue as well

Question 775

A bladder tumor that invades the submucosa is what stage?

Answer 775

T1

Question 776

A bladder tumor that invades the muscle layer of the bladder is what stage?

Answer 776

T2

Question 777

What is the treatment of BLADDER Tumors that invade the MUSCLE layer and beyond (T2-T4)?

Answer 777

RADICAL Cystectomy (bladder, adjacent pelvic organs, LN's) WITH urinary DIVERSION (bag, external catheter or intestine)

Question 778

How are Stage I BLADDER Cancer patients treated?

Answer 778

Trans Urethral Resection of the Bladder Tumor (TURBT)

Question 779

BLADDER Cancer Stages I (higher-risk) should be treated how?

Answer 779

With Trans Urethral Resection of the Bladder Tumor (TURBT) AND Intravesicular agents (bacillus Calmette-Guérin - 6 weeks induction and maintenance therapy x 1 YEAR)

Question 780

BLADDER Cancer Stages I (lower-risk) should be treated how?

Answer 780

With Trans Urethral Resection of the Bladder Tumor (TURBT) AND Observation OR SIGNLE-DOSE Intravesicular MITOMYCIN or GEMCITABINE

Question 781

How are patients with NON-invasive BLADDER Cancer followed post-treatment?

Answer 781

Cystoscopy every 3 MONTHS for the first 2 years, then every 6 MONTHS for the next 2 years, then yearly

Question 782

When is Chemotherapy and Radiation used for BLADDER Cancer?

Answer 782

When BLADDER-sparing treatment is possible

Question 783

What is the prognosis once BLADDER Cancer invades LN's/METS?

Answer 783

DISMAL (10-20% survival) - treat with PLATIN-based chemo for palliation

Question 784

What should be done if INVASIVE BLADDER Cancer is diagnosed at presentation?

Answer 784

PRE-OP NeoAdjuvant chemotherapy (PLATIN) and if not given, then give POST-OP (Adjuvant Chemotherapy)

Question 785

Where do most RENAL CELL cancers originate and what type are they histologically?

Answer 785

Renal CORTEX, of the "CLEAR-Cell" type (if originate in the renal pelvis - transitional cell type)

Question 786

What are the risk factors for RENAL CELL Cancer?

Answer 786

Smoking, Obesity, Cadmium, Gasoline, Asbestos, Dialysis

Question 787

What GENETIC (inherited) condition increases the risk for RENAL Cancer?

Answer 787

von Hippel-Lindau syndrome (pheochromocytoma, CNS tumors, STROKE, retinal angiomas, Chromosome 3 mutation)

Question 788

How are most RENAL CELL Cancers found?

Answer 788

Incidentally on Imaging

Question 789

What is the MOST important thing to determine after finding an INCIDENTAL renal mass on imaging?

Answer 789

Determine whether cystic or solid (by US)

Question 790

What should be done NEXT for a COMPLEX renal cyst or solid MASS seen on US?

Answer 790

CT scan

Question 791

When are CT-guided biopsies of RENAL SOLID masses done?

Answer 791

If they are SMALLER than 3 cm

Question 792

What is done for ANY SOLID RENAL mass ≥3-4 cm?

Answer 792

PARTIAL (if

Question 793

What should be done for RENAL masses ≤1.5 cm?

Answer 793

OBSERVATION

Question 794

What stage is a RENAL mass CONFINED to the KIDNEY that is ≤7 cm? What if >7 cm?

Answer 794

Stage I; Stage II

Question 795

What stage is a RENAL mass that extends into SURROUNDING tissues OR VASCULAR structures OR REGIONAL LN's?

Answer 795

Stage III

Question 796

How are Stage I-III RENAL CELL CANCERS treated?

Answer 796

PARTIAL (if

Question 797

How is Stage IV (METASTATIC) RENAL CELL CANCER treated?

Answer 797

NEPRECTOMY + IMMUNOTHERAPY (Interferon-alpha, Vascular Endothelial Growth Factor (VEGF) inhibitors - sunitinib, sorafenib, bevacizumab, temsirolimus, everolimus

Question 798

Patients with SPORADIC Clear-Cell RENAL Cancers (von Hippel-Lindau) or those with Stage IV (METASTATIC disease) are treated with what?

Answer 798

Vascular Endothelial Growth Factor (VEGF) inhibitors (sunitinib, sorafenib, bevacizumab, temsirolimus, everolimus)

Question 799

Where do RENAL CELL CANCES metastasize to?

Answer 799

Lung, Bone, Liver, Brain

Question 800

Firm and fixed lymph nodes imply what?

Answer 800

Malingnacy (lymphoma)

Question 801

What are the sites of LYMPHOID ORGANOMEGALY?

Answer 801

Spleen, Tonsills, Appendix, Thymus, Stomach

Question 802

When evaluating for LYMPHOMA, what tests may be helpful?

Answer 802

CBC w/diff, ESR, Peripheral Blood Smear, LDH, HIV, EBV (heterophile Ab), Hep B, C, CXR (hilar/mediastinal lymphadenopathy)

Question 803

What type of imaging after CXR can be helpful in deciding on biopsy sites when evaluating for LYMPHOMA?

Answer 803

CT, PET scans

Question 804

What types are MOST LYMPHOMAs?

Answer 804

Non-Hodgkin LYMPHOMAS

Question 805

This type of LYMPHOMA occurs most often in older MEN?

Answer 805

Non-Hodgkin LYMPHOMA

Question 806

What demographics are Hodgkin LYMPHOMAS seen in?

Answer 806

Younger patients with a BIMODAL distribution, 15-45 and >55

Question 807

Having a decreased immune function from HIV, HTLV-1, Hep C or EBV or from immunosuppressive drugs places one at greater risk for what type of LYMPHOMA?

Answer 807

Non-Hodgkin LYMPHOMA

Question 808

What type of LYMPHOMA is associated with chronic H.pylori inflammation?

Answer 808

MALT Lymphoma

Question 809

Exposure to HERBICIDES, CHLORINATED ORGANIC compounds and treatment for Hodgkin LYMPHOMA increases the risk of what malignancy?

Answer 809

Non-Hodgkin LYMPHOMA

Question 810

How should LN's be BIOPSIED in diagnosing LYMPHOMA?

Answer 810

ENTIRE LN BIOPSY, NO FNA!!!

Question 811

What tests are run on LN excisional biopsies in evaluation for LYMPHOMA?

Answer 811

MOLECULAR testing (cytogenetics, fluorescence in situ hybridization and gene expression), immunophenotyping and histopathology

Question 812

When is a Lumbar Puncture done for assessment of LYMPHOMA?

Answer 812

In patients at risk for CNS involvement (Non-Hodgkin LYMPHOMA involving the sinuses, testes, bone marrow or ocular sites)

Question 813

Mutations of genes in what LYMPHOCYTIC cells are associated with MOST Non-Hodgkin and Hodgkin LYMPHOMAS?

Answer 813

B-cells

Question 814

What LYMPHOMAS are considered INCURABLE?

Answer 814

LATE-Stage LYMPHOMAS and INDOLENT LYMPHOMAS

Question 815

How are "AGGRESSIVE" and "HIGHLY AGGRESSIVE" LYMPHOMAS treated?

Answer 815

Combination CHEMOTHERAPY

Question 816

LYMPHOMA features such as CD20 Ag expression and GERMINAL CENTER B-cell phenotype are considered what?

Answer 816

GOOD PROGNOSTIC indicators

Question 817

What CHEMOTHERAPEUTIC agent is used to treat LYMPHOMA?

Answer 817

RITUXIMAB (for BOTH initial and maintenance therapy)

Question 818

What treatment can be curative in LYMPHOMA besides COMBINATION CHEMOTHERAPY?

Answer 818

Allogenic Hematopoietic Stem Cell Transplantation (HSCT)

Question 819

What is the STANDARD for determining the EXTENT and STAGING of disease for Non-Hodgkin and Hodgkin LYMPHOMA?

Answer 819

Ann Arbor Staging System

Question 820

What is the LYMPHOMA (both Hodgkin & NHL) Stage when ONLY a SINGLE LN region/cluster OR a SINGLE EXTRALYMPHATIC site (tonsils, spleen, appendix, thymus, stomach)

Answer 820

Stage I

Question 821

What is the LYMPHOMA (both Hodgkin & NHL) Stage when TWO LN regions/clusters OR a LOCALIZED EXTRALYMPHATIC site (tonsils, spleen, appendix, thymus, stomach) ON THE SAME SIDE of the diaphragm?

Answer 821

Stage II

Question 822

What is the LYMPHOMA (both Hodgkin & NHL) Stage when involved LN regions/clusters on BOTH SIDES of the diaphragm ± SPLEEN involvement OR a LOCALIZED EXTRALYMPHATIC site (tonsils, spleen, appendix, thymus, stomach) OR BOTH?

Answer 822

Stage III

Question 823

What is the LYMPHOMA (both Hodgkin & NHL) Stage when there is MULTIFOCAL EXTRALYMPHATIC involvement ± LN involvement OR ISOLATED EXTRALYMPHATIC disease with DISTANT (non-local) LN involvement

Answer 823

Stage IV

Question 824

What is the HODGKIN LYMPHOMA (ONLY) Stage if disease is LIMITED to UPPER abdomen (spleen, splenic hilar, celiac or portahepatic nodes)?

Answer 824

Stage III-1

Question 825

What is the HODGKIN LYMPHOMA (ONLY) Stage if disease is LIMITED to LOWER abdomen (periaortic, pelvic or inguinal nodes)?

Answer 825

Stage III-2

Question 826

Histopathology, Immunotyping, Immunohistochemistry, Cytogenetic studies?

Answer 826

REQUIRED for diagnosis of LYMPHOMA

Question 827

This type of LYMPHOMA is HIHGLY CURABLE regardless of stage at presentation!!

Answer 827

HODGKIN LYMPHOMA

Question 828

Follicular LYMPHOMA, MALT LYMPHOMA, CLL and HAIRY CELL LEUKEMIA are what kind of LYMPHOMAS?

Answer 828

INDOLENT Lymphomas

Question 829

Of the INDOLENT LYMPHOMAS, which have a GOOD prognosis and are CURABLE without HSCT?

Answer 829

MALT Lymphoma and HAIRY CELL Leukemia

Question 830

Of the INDOLENT LYMPHOMAS which have a POOR prognosis and are INCURABLE except with HSCT?

Answer 830

FOLLICULAR Lymphoma and CLL

Question 831

Median age at diagnosis for this LYMPHOMA is 60, almost ALWAYS involves the BONE MARROW and involves the bcl-2 oncogene with a t(14;18) translocation, staging depends of SIZE of lymphocytes?

Answer 831

FOLLICULAR Lymphoma

Question 832

Which patients with the INDOLENT FOLLICULAR Lymphoma require EARLY and AGGRESSIVE therapy?

Answer 832

Stage III (LARGE-CELL LYMPHOMA) - large lymphocytes whereas stages I & II have "small lymphocytes"

Question 833

What should be done with patients found to have Stages I or II FOLLICULAR Lymphoma without symptoms?

Answer 833

DELAY treatment until symptoms or organ dysfunction (bone marrow involvement, lymphadenopathy) develop BECAUSE there is NO benefit with EARLY treatment

Question 834

What is the treatment for pts with FOLLICULAR Lymphoma with Stages I & II that are symptomatic or have organ dysfunction and for Stage III (large lymphocytes "large-cell lymphoma)?

Answer 834

``` RITUXIMAB-based (R-CHOP/R-CVP) R- RITUXIMAB C- Cyclophosphamide H- Doxorubicin ("Hydroxydaunomycin") O- Vincristine ("Oncovin") P- Prednisone ```

Question 835

What can be done as a last resort CURATIVE therapy for ALL LYMPHOMAS however with very high risk morbidity and mortality and usually reserved for young pts in otherwise good health?

Answer 835

Allogenic/Autologous Hematopoietic Stem Cell Transplantation (HSCT)

Question 836

B-cell origin (CD20-positive) LYMPHOMA associated with H.pylori which is curable WITHOUT chemotherapy but simply treating H.pylori?

Answer 836

MALT Lymphoma

Question 837

Rarely, this B-cell (CD20-positive) LYMPHOMA can have EXTRA-NODAL presentation with involvement of the COLON, LUNG, ORBIT, THYROID, SALIVARY GLANDS, BLADDER or primarily involve the SPLEEN and when not associated with the stomach or H.pylori, it is associated with Sjögren and Hashimoto Thyroiditis?

Answer 837

MALT Lymphoma

Question 838

How should MALT Lymphoma affecting the SPLEEN be treated?

Answer 838

SPLENECTOMY

Question 839

How should EXTRA-NODAL MALT Lymphoma (not associated with the stomach or H.pylori) be treated?

Answer 839

RADIATION Therapy + RITUXIBAM

Question 840

What is the most COMMON form of lymphoid malignancy?

Answer 840

CLL

Question 841

How do most CLL patients present?

Answer 841

ASYMPTOMATIC, found incidentally on lab work (LYMPHOCYTOSIS - Stage 1), Stage 2 - Lymphadenopathy, Stage 3 - Splenomegaly, Stage 4 - anemia/thrombocytopenia

Question 842

When should patients with CLL be treated?

Answer 842

When SYMPTOMATIC (fever, wt. loss, sweating, pain) or higher stages

Question 843

How is CLL treated?

Answer 843

RITUXIMAB or Fludarabine - based CHEMOTHERAPY

Question 844

CLL patients are at an increased risk of what due to their disease and treatment?

Answer 844

Opportunistic Infections - Pneumocystis jirovecii & Herpes

Question 845

What can you treat with if CLL undergoes TRANSFORMATION into secondary malignancies (LARGE-CELL Lymphoma - Stage III Folicular Lymphoma, or ProLymphocytic Leukemia)?

Answer 845

R-CHOP and HSCT

Question 846

A LEUKEMIA that is HIGHLY CURABLE although indolent that presents with PROGRESSIVE Cytopenia and Splenomegaly WITHOUT Lymphadenopathy?

Answer 846

HAIRY-CELL Leukemia

Question 847

What is HAIRY-CELL LEUKEMIA, a HIGHLY-CURABLE LEUKEMIA (although indolent) treated with?

Answer 847

CLADRIBINE

Question 848

An AGGRESSIVE LYMPHOMA of either B-cell (most common) or T-cell (CD4-associated) presenting with LYMPHADENOPATHY, fever, night sweats and weight loss?

Answer 848

DIFFUSE LARGE-CELL LYMPHOMA

Question 849

How is the AGGRESSIVE DIFFUSE LARGE-CELL (B-cell) LYMPHOMA treated?

Answer 849

R-CHOP + RADIATION Therapy

Question 850

How is the AGGRESSIVE DIFFUSE LARGE-CELL (T-cell) LYMPHOMA treated?

Answer 850

CHOP (without the RITUXIMAB)

Question 851

What is the MOST AGGRESSIVE LYMPHOMA?

Answer 851

Burkitt LYMPHOMA t(8:14)

Question 852

What should be done as soon as Burkitt LYMPHOMA is diagnosed?

Answer 852

IMMEDIATE treatment (life threatening)

Question 853

How is Burkitt LYMPHOMA treated?

Answer 853

R-EPOCH (RITUXIMAB based)

Question 854

LYMPHOMA found in the SMALL INTESTINE, COLON, BONE MARROW, PERIPHERAL BLOOD with an overexpression of cyclin D1 and a t(11;14) is diagnosed as what?

Answer 854

MANTLE-CELL LYMPHOMA (AGGRESSIVE)

Question 855

How is MANTLE-CELL LYMPHOMA treated?

Answer 855

RITUXIMAB-based CHEMOTHERAPY (R-CHOP, R-HYPERCVAD)

Question 856

What LYMPHOMA is HIGHLY CURABLE regardless of stage?

Answer 856

HODGKIN LYMPHOMA (although the higher the stage, the poorer survival)

Question 857

This LYMPHOMA usually presents with PALPABLE Lymphadenopathy or a MEDIASTINAL MASS that requires biopsy for diagnosis and is highly-curable?

Answer 857

HODGKIN LYMPHOMA

Question 858

How is HODGKIN LYMPHOMA treated if LOCALIZED disease only?

Answer 858

RADIATION Therapy

Question 859

How is HODGKIN LYMPHOMA treated at advanced (non-localized) stages?

Answer 859

ABVD - COMBINATION CHEMOTHERAPY

Question 860

For which HODGKIN LYMPHOMA Subtype, is RITUXIMAB added to the COMBINATION CHEMOTHERAPY?

Answer 860

B-cell (CD20)

Question 861

Mycosis Fungoides and Sézary syndrome are a type of LYMPHOMA that expresses the CD4 antigen and histologically feature a "CEREBRIFORM nucleus"?

Answer 861

Cutaneous T-CELL LYMPHOMA

Question 862

A LYMPHOMA that presents with Dry, Pruritic, Erythematous skin patches and plaques with diffuse erythema and ulcerated lesions with recurrent bacterial infections ans sepsis?

Answer 862

Cutaneous T-cell LYMPHOMA

Question 863

How are EARLY stages (limited cutaneous involvement) of Cutaneous T-cell LYMPHOMA treated? LATE stages?

Answer 863

Early - Prednisone and retinoid creams, P-UVA + Interferon-alpha Late - CHOP chemotherapy

Question 864

What should be biopsied in a patient presenting with a CANCER of UNKNOWN PRIMARY site?

Answer 864

Tumors from the most accessible locations

Question 865

Should an EXHAUSTIVE search for a PRIMARY tumor in an asymptomatic patient with a CANCER of UNKNOWN PRIMARY site be done?

Answer 865

NO! (does not improve outcome)

Question 866

Should general tumor markers or PET scans be done in pursuit of a CANCER of UNKNOWN PRIMARY site?

Answer 866

NO!

Question 867

How should testing be focused in pursuing a PRIMARY tumor in a patient with a CANCER of UNKNOWN PRIMARY site?

Answer 867

Symptom-Specific (if head/neck, abdominal, pelvic, etc.)

Question 868

What should you look for in a young patient presenting with a "poorly differentiated histologic findings of a tumor, part of a CANCER of UNKNOWN PRIMARY site, with centrally-located, symmetric retroperitoneal and mediastinal lymphadenopathy?

Answer 868

TESTICULAR GERM-Cell tumor (alpha-fetoprotein, ß-HCG, US of testes)

Question 869

How should patients with CANCER of UNKNOWN PRIMARY site be TREATED if no PRIMARY tumor is identified?

Answer 869

Site-specific (if suspicious for testicular cancer - platinum-based chemotherapy, if breast, head/neck, gastro, etc.)

Question 870

What is the difference in prognosis for a poorly differentiated carcinoma and an ADENOCARCINOMA of any differentiation?

Answer 870

Adenocarcinomas have the WORST prognosis

Question 871

A woman comes in with isolated axillary lymphadenopathy that is found to be MALIGNANT and it is therefore assumed that she has locoregional breast cancer, Breast MRI is done and is negative, whats the next step?

Answer 871

Treat as if Stage II Breast Cancer with MASTECTOMY and AXILLARY LN DISSECTION

Question 872

Sunburns during childhood, multiple cutaneous nevi are risk factors for?

Answer 872

MELANOMA

Question 873

What thickness in MELANOMA lesions determines PROGNOSIS?

Answer 873

1 mm (if 1 mm not as good, if LN involvement or metastatic, poor)

Question 874

How should be done for ALL melanomas?

Answer 874

SURGICAL resection (even for LIMITED metastatic disease that is surgically resectable)

Question 875

What should be done in ADDITION to SURGICAL resection for melanomas >1 mm thick?

Answer 875

SENTINEL LN Biopsy (if negative, no further surgery, if positive, LN dissection is recommended)

Question 876

How should MELANOMA patients be followed after SURGERY?

Answer 876

At LEAST ONCE ANNUALLY x 5 years with attention to local LN's

Question 877

What type of BRAIN tumor is a glioblastoma multiforme?

Answer 877

A grade IV anaplastic astrocytoma

Question 878

What is the treatment for glioblastoma multiforme?

Answer 878

Surgery with POST-OP whole-brain radiation

Question 879

What should be done after surgery for a glioblastoma multiforme?

Answer 879

WOLE-BRAIN Radiation (also done prophylactically for patients with SCLC)

Question 880

LUNG CANCER, also seen with B-cell lymphoma, Hodgkin Lymphoma or Germ-Cell Tumors (testicular) presenting with DYSPNEA, Facial swelling and EDEMA(plethora), UE edema and distended neck and chest veins have what?

Answer 880

Superior Vena Cava Syndrome (SVC syndrome)

Question 881

If a patient INITIALLY presents with DYSPNEA, Facial swelling and EDEMA(plethora), UE edema and distended neck and chest veins, they likely have what?

Answer 881

A previously-undiagnosed MALIGNANCY causing Superior Vena Cava Syndrome (SVC syndrome)

Question 882

How does Superior Vena Cava Syndrome (SVC syndrome) present on CXR?

Answer 882

Widened Mediastinum and Pleural Effusion

Question 883

What should be done for an INITIAL presentation of Superior Vena Cava Syndrome (SVC syndrome)?

Answer 883

Mediastinoscopy OR CT-guided tissue biopsy

Question 884

What is the treatment for Superior Vena Cava Syndrome (SVC syndrome)?

Answer 884

Treat underlying malignancy (COMBINED CHEMOTHERAPY and RADIATION Therapy)

Question 885

What cancers can cause brain mets with resulting increased intracranial pressure?

Answer 885

Lung, Melanoma

Question 886

Lung cancer or melanoma p/w persistent HA, vomiting, AMS or focal neurologic deficit should alert you of what possibility?

Answer 886

Brain mets with increased intracranial pressure

Question 887

What SHOULD be done and what should NOT be done if suspecting increased intracranial pressure from cancer mets to the brain?

Answer 887

SHOULD - Immediate CT/MRI, DEXAMETHASONE ± MANNITOL (diuretic) Should NOT - DO NOT DO LP - brainstem herniation

Question 888

When should you NOT start steroids for brain CANCER prior to obtaining a tissue BIOPSY?

Answer 888

If suspecting a PRIMARY CNS LYMPHOMA

Question 889

How is OBSTRUCTING HYDROCEPHALUS treated when causing increased intracranial pressure?

Answer 889

SURGICAL Drainage/shunt placement

Question 890

What should be suspected if a patient with either BREAST, LUNG or PROSTATE Cancer presented with localized BACK pain with a heaviness and weakness in the legs with difficulty climbing stairs or getting up from a seated position as well as c/o bowel/bladder dysfunction?

Answer 890

SPINAL CORD COMPRESSION

Question 891

Besides a physical exam (pain with palpation, hyperreflexia, decreased LE muscle strength) what else should be done for a patient who presents with symptoms suspicious for spinal CORD compression?

Answer 891

MRI of the ENTIRE spine

Question 892

How is SPINAL CORD COMPRESSION treated?

Answer 892

DEXAMETHASONE + RADIATION Therapy or NEUROSURGERY if severe

Question 893

What does a MALIGNANT pleural effusion indicate?

Answer 893

An undiagnosed LUNG or BREAST cancer or a LYMPHOMA, usually at an advanced, incurable stage

Question 894

What should be done if a MALIGNANT pleural effusion is suspected?

Answer 894

CXR/CT scan and THORACENTESIS for fluid sample and immediate drainage palliation

Question 895

How much fluid can be safely drained from a pleural effusion before causing pulmonary edema?

Answer 895

≤1500 ml

Question 896

Since 70% of MALIGNANT pleural effusions recur, what is done to prevent recurrence?

Answer 896

Chest-Tube drainage + Pleurodesis (tetracycline, bleomycin or talc)

Question 897

A patient presents with a h/o LUNG or BREAST cancer or a LYMPHOMA with CHEST PAIN, low voltage QRS on ECG with dyspnea, orthopnea and hypotension with a CXR suggesting an enlarged heart, what is suspected?

Answer 897

Pericardial EFFUSION

Question 898

What should be done for a symptomatic MALIGNANT pericardial effusion (slow growing)?

Answer 898

ECHO to confirm, then SUBXIPHOID cardiocentesis with immediate drainage followed by partial pericardiectomy or pericardial window placement for long-term relief

Question 899

What should be suspected in a patient with LARGE-Cell Leukemia, CLL, Burkitt Lymphoma or someone whose cancer was very sensitive to chemotherapy and presented with HYPERkalemia, HYPERphosphatemia, HYPERuricemia HYPOcalcemia, DIC and ACUTE Kidney Failure?

Answer 899

TUMOR LYSIS SYNDROME (EMERGENCY)

Question 900

How is Tumor Lysis Syndrome treated?

Answer 900

IVF w/NS + Allopurinol/RASBURICASE (to rapidly decrease hyperuricemia) ± Dialysis (for uremia, hyperkalemia, hyperphosphatemia)

Question 901

Pt presents with NAUSEA/VOMITING, CONSTIPATION, POLYURIA, POLYDIPSIA, WEAKNESS and CONFUSION and has a history of MULTIPLE MYELOMA (or breast, kidney or lung cancer) should be suspected of having what?

Answer 901

HYPERCALCEMIA

Question 902

Wha tis hypercalcemia due to in association with malignancy?

Answer 902

Direct bone destruction as well as parathyroid-hormone related protein released by malignant cells

Question 903

How is HYPERCALCEMIA due to malignancy treated?

Answer 903

IVF with NS + BISPHOSPHONATES (pamidronate/zolendronic acid) + STEROIDS (if multiple myeloma)

Question 904

Below what level of ABSOLUTE NEUTROPHILS is there a risk of life threatening infection (can be seen with chemotherapy regimens)?

Question 905

How is life-threatening Neutropenia treated in the setting of chemotherapy that must be continued?

Answer 905

With Granulocyte Colony Stimulating Factors G-CSF) and Granulocyte-Macrophage Colony Stimulating Factors (GM-CSF)

Question 906

How should patients with NEUTROPENIA who develop fever be treated?

Answer 906

BROAD SPECTRUM ANTIBIOTICS (CEFtazidime, CEFepime, meropenem, imipenem, piperacillin-tazobactam)

Question 907

What treatment does PROLONGED NEUTROPENIA following INDUCTION CHEMOTHERAPY for AML REQUIRE?

Answer 907

EMPIRIC ANTIFUNGAL Therapy (Amphotericin B)

Question 908

What formulation of anthracyclines is LESS cardiotoxic (LV dysfunction)?

Answer 908

LIPOSOMAL (liposomal doxorubicin, etc.)

Question 909

Cardiotoxicity with which CHEMOTHERAPEUTIC agent is reversible when the agent is stopped?

Answer 909

Trastuzumab (used for HER2/neu inhibition)

Question 910

What CHEMOTHERAPEUTIC agent has been associated with PNEUMONITIS and MUST be discontinued when ≥25% reduction in DLCO is seen and treated with STEROIDS?

Answer 910

BLEOMYCIN

Question 911

What cancer therapy can induce pneumonitis when there is a rapid withdrawal of CORTICOSTEROIDS and the treatment is restarting corticosteroids with TAPERED withdrawal?

Answer 911

RADIATION Therapy

Question 912

What chemotherapeutic agent causes hemorrhagic cystitis?

Answer 912

CYCLOPHOSPHAMIDE

Question 913

What ADVERSE EVENT do the agents CISPLATIN and METHOTREXATE cause? How is it treated?

Answer 913

RENAL tubular toxicity; treated with IVF and forced diuresis

Question 914

How is sexual dysfunction treated in CHEMOTHERAPY?

Answer 914

phosphodiesterase-5 enzyme inhibitors (men), low-dose topical estrogens (women)