Hematology

Question 1

Jak2 V617F mutation that have essential thrombocytopenia, what is the best treatment regimen?

Answer 1

Aspirin and Hydroxyurea

Question 2

What heme/onc medication can reduce neutrophils?

Answer 2

Methotrexate

Question 3

IgG monoclonal antibody spike, everything else is normal, what else should be done?

Answer 3

Nothing, observation, MGUS, no follow up testing required.

Question 4

Sickle cell patient needs gallbladder removal, what should be the hemoglobin goal?

Answer 4

Pre-surgery, hemoglobin above 10

Question 5

A patient has parvo-virus s/p chemo, and she is a Kindergarten teacher. What is the next best treatment if reticulocyte count is low?

Answer 5

IV immunoglobulin

Parvovirus can lead to red cell aplasia

Question 6

If a patient has CAD and a GI bleed, they take Plavix and aspirin on a routine basis. Hemoglobin 10, platelets are wnl. What should be next best step?

Answer 6

Hold plavix, give ASA, call cardiology

Question 7

If a patient has a normal hemoglobin electrophoresis, a thalassemia is suspected, what is the most likely concern?

Answer 7

Alpha Thalassemia

Question 8

C282Y, H63D, and 65C mutations are the most common mutations with what liver pathology?

Answer 8

Hemochromatosis

Question 9

If all three cell lines are low, no blast cells are seen, what is the most likely heme pathology?

Answer 9

Myelodysplastic Syndrome (Myelodysplasia)

Question 10

What is a very common cause of neutrophilia?

Answer 10

Chronic Smoking

Question 11

Superficial vein thrombosis usually has AC treatment for how long?

Superficial vein thrombosis greater than 5cm should have treatment for how long?

Answer 11

6 weeks

3 months

Question 12

If a patient has an elevated INR less than 10 and they are asymptommatic, what should be done?

Answer 12

Watch and wait

Question 13

If a patient has an elevated INR greater than 10 and no bleeding, what should be given?

Answer 13

Vitamin K

Question 14

If a patient has an elevated INR greater than 10 and bleeding, what should be given?

Answer 14

Vitamin K and Kcentra (4-factor prothrombin complex concentrate)

Question 15

If a patient has unexplained ITP, what are the first things to test for?

Answer 15

Hep C and HIV

Question 16

Pediatric Leukemia patients that have had treatment are at an increased risk of what?

Answer 16

Diabetes Mellitus and HLD

Question 17

If a patient is starting chemotherapy, what should be given to them to maintain neutrophil count?

Answer 17

Granulocyte-CSF, helps preserve WBC count

Question 18

Warm autoimmune hemolytic anemia obscures detection of what?

Answer 18

Alloantibodies, complicating identification of compatible donors

Question 19

Warm autoimmune hemolytic anemia, how does one eliminate antibody production?

Answer 19

Glucocorticoids and Rituximab

Question 20

A patient has hereditary spherocytosis, mild symptoms, what should be done?

Severe Symptoms, what should be done?

Answer 20

Folate

Splenectomy

Question 21

If a patient has a central venous catheter placed, clot forms, and the central venous catheter is still functioning, what should be done?

Answer 21

Keep the central venous catheter in place, and AC for three months

Question 22

If a patient is low risk and he has multiple subsegmental PE, what should be done?

Answer 22

D/C home with Rivaroxaban

Question 23

A patient has G6PD deficiency, no acute symptoms, what is the best treatment course?

Answer 23

Symptom management

Question 24

Are steroids used in DIC treatment?

What is the treatment?

Answer 24

No

Treat the underlying cause

Question 25

A patient has platelets less than 50, what should be done? If a patient requires a platelet transfusion, how much should the platelet count go up?

Answer 25

Transfusion Should go up 20,000 to 30,000

Question 26

A bone marrow biopsy shows marked hypocellularity, increased fat content without dysplastic cells, overall work up is negative, what is this? Patient is younger than 50, what is a possible treatment?

Answer 26

Aplastic anemia Bone Marrow Transplant

Question 27

What is the aplastic anemia treatment for older than 50, no suitable stem cell donor?

Answer 27

Immunosuppression with anti-thymocyte globulin, eltrombopag, cyclosporine, and prednisone

Question 28

Azacytidine or Decitabine can be used in what heme/onc disease that can help decrease transfusion dependence?

Answer 28

Myelodysplastic Syndrome

Question 29

Antiphospholipid syndrome has what unique clotting pattern?

Answer 29

Arterial and Venous embolism

Question 30

What is the drug of choice for antiphospholipid syndrome??

Answer 30

Warfarin, should start with heparin first Particularly triple positive lupus patients

Question 31

A patient takes a macrolide and has hemolytic anemia. What is the best test to run?

Answer 31

Cold agglutin Titer

Question 32

Thrombocytopenia and microangiopathic hemolytic anemia, easy bruising, what is the best initial treatment?

Answer 32

Plasma Exchange, Glucocorticoids, and Rituximab

Question 33

What enzyme is missing in the TTP?

Answer 33

ADAMS13

Question 34

If a patient has a PESI score of 1 with a pulmonary embolism, what is the best treatment?

Answer 34

Apixaban or Elliquis

Question 35

In a patient with an unprovoked DVT, how long should the treatment course be?

Answer 35

3-6 months, can be reduced after a discussion

Question 36

Does pregnancy naturally have an elevated D-dimer? What scoring criteria is used to evaluate if a pregnant woman should undergo radiological testing?

Answer 36

Yes YEARS algorithm

Question 37

Transfusion-associated circulatory overload occurs when? What is it?

Answer 37

6 hours Respiratory Distress, positive fluid balance, pulmonary edema

Question 38

How is the difference between Gestational Thrombocytopenia and ITP found?

Answer 38

Gestational Thrombocytopenia: later in pregnancy, platelets around 100,000 ITP: Early in pregnancy, platelets around 70,000

Question 39

A sickle cell patient with fever, hypoxia, chest pain, and pulmonary edema, would benefit from what treatment?

Answer 39

Erythrocyte Exchange Transfusion

Question 40

Platelet Transfusion does not normally occur in thrombocytopenia unless the patient is bleeding and below what number?

Answer 40

10-20k

Question 41

If a patient has platelets less than 30k and no active bleeding, what treatment is indicated?

Answer 41

Steroids

Question 42

Hemophilia A has what inheritance pattern? Who will be affected?

Answer 42

X-linked recessive men only are affected, women are asymptomatic

Question 43

If a patient is undergoing Multiple Myeloma Treatment with proteasome inhibitors, what other medications should be given?

Answer 43

Valacyclovir, helps any viral/varicella reactivation occurrence

Question 44

Patients with unexplained splanchnic vein thrombosis, what should be tested?

Answer 44

JAK2 mutation

Question 45

Normocytic anemia, what are the next best tests to order?

Answer 45

Reticulocyte Count and peripheral blood smear

Question 46

The first step in evaluating thrombocytopenia is what?

Answer 46

Peripheral Blood Smear

Question 47

Immune Mediated hemolysis is detected by what?

Answer 47

Positive Direct antiglobulin test

Question 48

If a patient has a chemoradiation due to breast cancer, and they have myelodysplastic syndrome. What is the next best test?

Answer 48

Bone Biopsy

Question 49

Hydroxyurea treatment can inadvertently cause what?

Answer 49

Macrocytic Anemia

Question 50

AML in a 79 year old woman, she responds well to induction therapy, what is the next best consolidation therapy step?

Answer 50

Allogenic Hematopoietic Stem Cell Transplantation

Question 51

Patient most likely has Heparin Induced Thrombocytopenia, what is the next best treatment step? Assuming she needs to be anticoagulated

Answer 51

Start Rivaroxaban

Question 52

Polycythemia Vera, patient under 60, and low risk, what should be done? Polycythemia Vera, patients over 60, and high risk, what should be done?

Answer 52

Low Risk patient: Low Dose Aspirin and phlebotomy High Risk patient: Hydroxyurea or IFN-alpha, and phlebotomy

Question 53

Before starting chemotherapy and managing VTE, what should be done to assess the need for DVT prophylaxis?

Answer 53

Khorana Score

Question 54

Dabigatran causes a large hemorrhagic bleed, what is the reversal agent?

Answer 54

Idarucizumab

Question 55

A whole-body low-dose CT scan is negative for smoldering myeloma, what is the next best step?

Answer 55

Whole Body MRI

Question 56

Elevated IgM, hyperviscosity, difficulty with vision, fever, night sweats, weight loss, what is the next treatment step?

Answer 56

Plasmapharesis

Question 57

B-thalassemia patients should be heavily monitored for what?

Answer 57

iron overload

Question 58

All multiple myeloma patients should have what treatment done?

Answer 58

Pneumococcal Vaccination

Question 59

Anemia of chronic disease, what is the best treatment course?

Answer 59

Reverse the original cause and give steroids

Question 60

In liver disease vs. DIC, what clotting factor should be measured?

Answer 60

Factor VIII

Question 61

Amyloidosis can be confirmed by a biopsy located where?

Answer 61

Abdominal Fat Pad

Question 62

Elevated aPTT, what is the best step to evaluate the cause?

Answer 62

Mixing Study

Question 63

Warm autoimmune hemolytic anemia is best detected how?

Answer 63

Positive Direct antiglobulin test that detects IgG and C3

Question 64

A patient receives chimeric T cell therapy, patients with a high disease burden may develop what?

Answer 64

Cytokine Release Syndrome

Question 65

Proximal Deep venous clot treatment with ESRD on dialysis, is what?

Answer 65

Apixaban (Eliquis), lowest renal elimination Rivaroxaban is eliminated through the kidneys

Question 66

What is this?

Answer 66

Iron Deficiency Anemia

Question 67

What is this?

Answer 67

Normal Heme Path Slide

Question 68

What is this?

Answer 68

Normal packed RBCs

Question 69

What is the black arrow pointing at?

Answer 69

Iron deficiency anemia

Question 70

What two pathologies are on this slide?

Answer 70

Target Cell and Thalassemia

Question 71

What is this?

Answer 71

Warm IHA

Question 72

Autoimmune, lymphoproliferative disorders, B-cell non-Hodgkin lymphoma, and drug induced problems can cause what type of anemia?

Answer 72

Warm Autoimmune Hemolytic anemia

Question 73

Here/Onc: Autoimmune, what process is typically infectious?

Answer 73

Cold Autoimmune Hemolytic Anemia

Question 74

Cold agglutinin disease can be treated how?

Answer 74

Rituximab

Question 75

CLL, what feature (Path slide) will be there for a diagnosis? CLL, if having recurrent infections, what should be considered?

Answer 75

Smudge Cell IVIG

Question 76

What is this? What CD numbers will be positive for this pathology?

Answer 76

CLL CD5, CD20, CD23

Question 77

What is this? What is the distinguishing feature? What is the first line treatment?

Answer 77

CLL, smudge cell Ibrutinib, acalabrutinib, and zanubrutinib

Question 78

What is this?

Answer 78

ALL, note, no myeloid or granules in the picture

Question 79

What is this?

Answer 79

Multiple Myeloma

Question 80

What is this?

Answer 80

AML, look at the auer rods

Question 81

What is this?

Answer 81

CML Philadelphia Chromosome t9:22

Question 82

What cell shows up in a CML smear slide?

Answer 82

Basophils are heavy, slide will show multiple different myelocyte phases

Question 83

What is this?

Answer 83

TTP, low platelet with AIHA

Question 84

What is this? What are the black arrows pointing at?

Answer 84

Schistocytes

Question 85

What is this? What can be see on the pathology slide?

Answer 85

ITP, no platelets around

Question 86

What is this?

Answer 86

Essential Thrombocytosis

Question 87

Increased reticulocyte count (RI > 2%), increased LDH, decreased haptoglobin, increased indirect bilirubin, what is this?

Answer 87

Hemolytic Anemia

Question 88

Extravascular hemolytic anemia has what issue?

Answer 88

Splenomegaly

Question 89

What syndrome has acquired clonal stem cell disorder, inactivating somatic mutation of PIG-A gene deficiency of GPI-anchor of CD55 and CD59, poor complement inhibition? Dark urine is seen in the morning, sometimes

Answer 89

Paroxysmal nocturnal hemoglobinuria (PNH)

Question 90

What is one of the treatments for symptomatic paroxysmal nocturnal hemoglobinuria?

Answer 90

Eculizumab or ravulizumab

Question 91

Symptomatic paroxysmal nocturnal hemoglobinuria is what type of hemoglobin disorder?

Answer 91

Myelodysplastic Syndromes

Question 92

G6PD has what issue?

Answer 92

X-linked defect of metabolism, susceptibility to oxidative damage

Question 93

Sulfonamides Dapsone Nitrofurantoin Rasburicase Primaquine Doxorubicin Methylene Blue INFX, DKA, fava beans can cause what?

Answer 93

G6PD Deficiency

Question 94

Bite Cells show up in what problem?

Answer 94

G6PD Deficiency

Question 95

Sickle Cell, what can be given to prevent a pain crisis?

Answer 95

L-glutamine

Question 96

Sickle Cell, pain, and vaso-occlusive crises, what is the =mab?

Answer 96

Crizanlizumab

Question 97

What type of infections are particularly scary with sickle cell?

Answer 97

Osteomyelitis, encapsulated organisms

Question 98

Hereditary Spherocytosis has a defect in what?

Answer 98

Ankyrin, alpa and beta spectrin, band 3

Question 99

Osmotic Fragility Test and low Eosin-5-maleimide binding is code for what hematology problem?

Answer 99

Hereditary Spherocytosis

Question 100

Warm Autoimmune Hemolytic Anemia is caused by what?

Answer 100

Idiopathic, CLL, NHL (Non-hodgkin lymphoma), autoimmune (SLE), HIV, Babesiosis, and drugs

Question 101

Cold Autoimmune Hemolytic Anemia is caused by what?

Answer 101

Intravascular hemolysis and acrocyanosis, Lymphoprolif. disorders (Waldenstroom's monoclonal), Mycoplasma Pneumoniae and EBV

Question 102

Warm AIHA treatment is what?

Answer 102

Corticosteroids, splenectomy, IVIG, Cytotoxic agents, rituximab

Question 103

Cold AIHA treatment is what?

Answer 103

Corticosteroids and rituximab

Question 104

Low ADAMTS13 is code for what disease?

Answer 104

Thrombotic Thrombocytopenic Purpura (TTP)

Question 105

Burr cell (Echinocytes) is seen in what disease?

Answer 105

Uremia and MAHA

Question 106

Acanththocytes (Spur cells) are seen in what disease?

Answer 106

Severe Liver Disease

Question 107

Target cells are seen in what 3 diseases?

Answer 107

Liver Disease, Thalassemia, HbC disease

Question 108

# What is this? Tear Drop RBCs with nucleated RBCs and immature WBCs, a dry tap (via bone biopsy), time course is a few months? Pathology slide is attached

Answer 108

Primary Myelofibrosis Green arrow is a tear drop erythrocyte Blue arrow is a nucleated RBC If symptomatic, stem cell transplant is only curative treatment

Question 109

Paroxysmal nocturnal hemoglobinuria (PNH), what is a good first line treatment? Not a -mab

Answer 109

Cyclosporine and anti-thymocyte globulin

Question 110

Venetoclax is a new first line treatment for what disease?

Answer 110

CLL

Question 111

A patient with a low-risk Myelodysplastic Syndrome, 5q cytogenic abnormality, can be given what drug if they are transfusion dependent?

Answer 111

Lenalidomide

Question 112

A patient with Myelodysplastic Syndrome can have a transformation into what concerning cancer?

Answer 112

AML transformation

Question 113

What heme/onc disease mechanism is bad hematopoiesis leading to hypercellular bone marrow and peripheral cytopenias (RBC, WBC, and Platelets do not function well)? Hint: look at path slide

Answer 113

Myelodysplastic Syndrome Patient has minimal platelets, nucleated RBC, and dysplastic neutrophil (picture from MKSAP)