Heme/Onc Flashcards

1
Q

Patient presents with what looks like anemia. What is the next step in diagnosis?

A

Complete blood count to look at hemoglobin, hematocrit and mean corpuscular volume

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2
Q

what does labs of anemia due to hemolysis look like?

A
Normocytic anemia (85-100)
Elevated bilirubin (normal <1)
Elevated LDH (normal<250)
Low haptoglobin (normal 50- 150)
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3
Q

What is pernicious anemia?

A

In pernicious anemia, there is either:

1) antibodies against parietal cells destroy the cells that produce intrinsic factor or
2) there is antibody inactivation of intrinsic factor

Either way this leads to deficiency of IF–> B12 deficiency

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4
Q

What is the Go to test for microcytic anemia?

A

Ferritin-

Elevated in anemia of chronic disease

Low in iron deficiency anemia

Normal in thalassemia

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5
Q

Thalassemia with normal hemoglobin electrophoresis is…

A

alpha thalassemia because if alpha is defective and whatever alpha is made is still able to pair with normal beta chain.

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6
Q

Elevated iron in a microcytic anemia means..

A

Sideroblastic anemia (could be part of developing malignancy) Get bone marrow biopsy to confirm.

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7
Q

Examples of diseases causing normocytic anemia (MCV 85-100)?

A

1) Hereditary Spherocytosis (hemolytic)
2) Anemia of Acute blood loss
3) Paroxysmal Nocturnal Hemoglobinuria (hemolytic)
4) G6PD Deficiency (hemolytic)
5) Sickle Cell (hemolytic)

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8
Q

What kind of anemia does Paroxysmal nocturnal hemoglobinuria cause?

A

Normocytic Hemolytic anemia caused by deficiency in the PIGA gene—
GDI anchor fails and RBCs become susceptible to endogenous complement fixation.

Dark urine, usually upon awakening (due to hemosiderin), abdominal pain

Diagnose w/ Flow cytometry looking for deficiency of CD55 & CD59

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9
Q

Labs of a hemolytic anemia will show:

A

Elevated bilirubin (normal < 1)

Elevated reticulocytes ( Greater than 2%)

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10
Q

Treatment for hereditary spherocytosis?

A
  • Spherocytes on smear
  • Positive osmotic fragility test
  • Treat mild disease with vitamins ( Folate)
  • Severe anemia– splenectomy
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11
Q

Pathophysiology of hereditary spherocytosis cause?

A

Caused by deficient red blood cell membrane protein such as spectrin, Ankyrin, or band 4.1 which causes weird shaped RBC to get stuck in spleen.

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12
Q

What is the test of choice for a non-tender lymph node?

A

If you suspect lymphoma- Do excisional biopsy

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13
Q

Four stages of lymphoma:

A

Stage 1-single node

Stage 2- Multiple nodes on the same side of the diaphragm

Stage 3- Multiple nodes on both sides of the diaphragm

Stage 4- Disseminated disease; marrow or blood

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14
Q

How to diagnose Hodgkins lymphoma?

A

Excisional biopsy will show Reed sternberg cells (+ for CD15 and CD30)

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15
Q

What is the treatment for Non-Hodgkins lymphoma/ B cell lymphoma)?

A

Patients w/ stage 2A or better get radiation

Patients w/ stage 2B (B symptoms) or worse get CHOP- R:

cyclophosphamide, vincristine & prednisone w/ rituximab

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16
Q

What is the treatment for Hodgkins lymphoma?

A

ABVD

Adriamycin/Doxorubicin, Bleomycin, vinblastine, dacarbazine)

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17
Q

What is multiple myeloma?

A

Plasma cell dysfunction making Immunoglobulins against phantom antigen (useless)

So pt gets Recurrent infections because they have all this useless antibodies

CRABS- Hypercalcemia, Renal failure, Anemia, Bone lytic lesions

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18
Q

How do you diagnose multiple myeloma?

A

Bone marrow biopsy is required to definitively diagnose MM

Hgb electrophoresis (Spep) shows M spike

Urine electrophoresis (upep) will show bence jones protein

Elevated protein gap

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19
Q

What is the treatment for multiple myeloma?

A

Everyone gets Chemo- Thalidomide or Bortezomib + Melphalan & prednisone

Age < 70 Also do stem-cell transplant

Age> 70 or no donor then just chemo

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20
Q

What is Monoclonal gammopathy of undetermined significance (MGUS)?

A

No anemia, no renal failure, no hypercalcemia, or bone pain. Only plasmacytosis of <10% but with (+) M spike.

Also (-) no bence jones protein on Upep

Surveillance q6months with labs to catch possible transformation to MM.

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21
Q

What is Waldenstrom Macroglobulinemia?

A

Lots of IgM (pentad) production which accumulates and causes 1) Peripheral neuropathy and 2) hyperviscosity syndrome

Elevated M-spike on Spep

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22
Q

How is Waldenstrom Macroglobulinemia diagnosed?

A

Elevated M-spike on Spep

Bone marrow biopsy > 10% lymphoplasmacytic lymphoma

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23
Q

What is the treatment for Waldenstrom Macroglobulinemia?

A

Plasmapheresis for hyperviscosity

Chemo w/ Rituximab regimen + haematopoietic stem cell transplant (HSCT)

No treatment if asymp and elderly

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24
Q

What is the treatment for Von willebrand disease?

A

DDAVP

Desmopressin exerts its haemostatic effect by:

1) Inducing synthesis of the von Willebrand factor (VWF) by endothelial cells.
2) Stimulating release of the VWF from its storage sites in endothelial cells.
3) Cleaving the large VWF multimers circulating in plasma into smaller multimers.

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25
Q

Indications for transfusion of platelets?

A

<20,000 – to reduce risk of spontaneous hemorrhage

<50,000 AND bleeding

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26
Q

What is the FASTEST way to reverse coumdin/ warfarin effects?

A

Fresh frozen plasma

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27
Q

What is the treatment for TTP?

A

Plasmapheresis

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28
Q

How do you treat Heparin induced thrombocytopenia (HIT)?

A

Stop all heparin products

Use lepirudin to treat

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29
Q

What is Thrombotic Thrombocytopenic Purpura (TTP)?

A

ADAMTS13 deficiency

Fever 
Anemia (microangiopathic hemolytic anemia presenting with severe fatigue)
Thrombocytopenia 
Renal 
Neuro Symptoms 

3 or more of this pentad with schistocytes on the blood smear and a normal INR/PTT, they likely have TTP.

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30
Q

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

A

avoid transfusing platelets if possible–> may worsen clots

Need plasma exchange/plasmapheresis which works to filter circulating antibodies against ADAMTS13

Transfusion of FFP can be helpful if plasmapheresis not immediately available

31
Q

Diagnosis of Choriocarcinoma

A

Most aggressive type of germ cell tumor

beta-hCG >1000 IU/L

32
Q

Diagnosis of Yolk sac tumors

A

Most common testicular malignancy in prepubertal males

AFP is >100 ng/mL

33
Q

Diagnosis of Seminoma

A

Germ cell tumor that is a well-defined hypoechoic mass

Moderate elevation of beta-hCG

34
Q

Staging for CLL

A

Rai and Binet staging systems

35
Q

What is Behçet syndrome?

A

Recurrent aphthous ulcers, genital ulcerations, and uveitis leading to blindness

Treat acute exacerbations of Behçet syndrome with oral prednisone.

36
Q

What is Tumor lysis syndrome?

A

Results when the intercellular contents of tumor cells are released into the serum during chemotherapy producing hyperkalemia, hyperuricemia, hyperphosphatemia, and secondary hypocalcemia.

37
Q

How to prevent Tumor lysis syndrome?

A

Prevention:

1) IV hydration
2) Hypouricemic agents such as allopurinol, rasburicase, or febuxostat

38
Q

Transitioning from insulin drip to subQ

A

When the patient is ready to transition off the insulin drip for diabetic ketoacidosis (DKA), start subcutaneous insulin BEFORE turning off the insulin drip to prevent rebound DKA.

39
Q

What is fever of unknown origin?

A

Fever of unknown origin (FUO) is a term used to describe prolonged fevers of >38.3°C (101°F) with no established diagnosis despite a thorough evaluation. The leading causes of FUO include collagen vascular diseases, infections, and malignancies.

40
Q

Mechanism of Anterior cruciate ligament tear

A

Anterior cruciate ligament tears are common among running and jumping athletes. They most commonly occur from a non-contact cutting movement, where the patient experiences a “pop” or giving way episode with immediate pain and rapid swelling. The most common mechanism of injury occurs during a cutting movement with the foot planted and tibia externally rotated with a valgus force across the knee, buckling it inward toward mid-line, forcing the tibia anteriorly.

41
Q

Mechanism of posterior cruciate ligament tear

A

PCL tears are usually due to a direct blow to the anterior tibia, forcing the tibia posteriorly and tearing the PCL fibers. The PCL acts as the primary restraint to posterior translation of the tibia. Patients with a PCL tear will present in the same manner as this patient, with deep knee pain, effusion, and decreased range of motion, but the mechanism of injury provides important information as to the structure involved. A positive posterior drawer test provides sufficient evidence to diagnose a PCL tear. This is confirmed with an MRI.

42
Q

Antibiotics in neutropenic fever

A

All patients with neutropenic fever should receive immediate treatment with an antibiotic that is broad spectrum and covers gram-positive and gram-negative organisms including Pseudomonas. Such antibiotics include piperacillin-tazobactam, meropenem, imipenem-cilastatin, and cefepime.

43
Q

Granulomatosis with polyangiitis (Wegener disease)

A

Common vasculitis that presents with upper respiratory lesions, alveolar hemorrhage, glomerulonephritis

Cytoplasmic antineutrophil cytoplasmic antibody (C-ANCA) and antiproteinase-3 antibody.

44
Q

Sturge-Weber syndrome

A

Congenital vascular disorder affecting the capillaries. Affects the young

Port-wine stain in the distribution of the trigeminal nerve.

Common complications include seizure, intellectual disability, and glaucoma.

45
Q

Polyarteritis nodosa

A

p-ANCA

Positive hepatitis B surface antigen or antibody

Mononeuropathies such as a foot drop.

46
Q

Goodpasture syndrome

A

Pulmonary-renal syndrome

Antiglomerular basement membrane antibody

Typically does not involve the upper respiratory symptoms

47
Q

Churg-Strauss syndrome

A

Positive peripheral cytoplasmic antibody (p-ANCA)

History of asthma

Eosinophilia

48
Q

Treatments and cure for paroxysmal nocturnal hemoglobinuria (PNH)

A

Treatment: Prednisone and Eculizumab

Cure: Allogeneic hematopoietic stem cell transplantation

49
Q

Peutz-Jeghers syndrome

A

Autosomal dominant mutation in a tumor suppressor gene (STK11)

hamartomatous polyps of the gastrointestinal tract; mucocutaneous hyperpigmentation of the mouth

50
Q

Patients of Peutz-Jeghers syndrome are at increased risk of what cancers?

A

Start screenings at an early age

Gastrointestinal: 
Colorectal (39%)
Gastric (29%)
Small bowel (13%)
Pancreatic (11%-36%)
Extraintestinal: 
Breast (32%-54%)
Ovary (21%)
Cervix (10%)
Sertoli cell tumor of the testis (9%)
Lung
Thyroid
51
Q

Key words and diagnosis

A

Severe aplasia- sickle cell aplastic crisis

Blast cells with Auer rods- acute promyelocytic anemia

“Dry tap”- primary myelofibrosis

Reed sternberg cells- Hodgkins Lymphoma

52
Q

Immune thrombocytopenic purpura (ITP)

A

Disease of peripheral platelet destruction by circulating autoantibodies

Look for pt presenting weeks after viral infection with nonblanching rash

Stable pt- corticosteroids and IVIG

Unstable- Platelet transfusion

Refractory pts- splenectomy

53
Q

Hepatic Adenoma

A

Encapsulated, well circumscribed mass with central necrosis and calcifications on a computed tomography (CT) scan

commonly found in Young, childbearing aged women
Associated with OCPs and anabolic steroids

54
Q

Treatment for Hepatic adenoma

A

Hepatic adenomas are benign epithelial tumors that have a risk of malignant transformation and rupture.

Patients should stop taking oral contraceptives, and large or symptomatic tumors should be surgically resected.

55
Q

Von Willebrand disease (VWD)

A

Most common inherited bleeding disorder, is an autosomal dominant condition

Low levels of factor VIII because vWF carries factor VIII, and without vWF, factor VIII is easily degraded.

Nosebleeds, oral bleeding, menorrhagia, easy bruising, or postincisional bleeding.

56
Q

Diagnosis of Von Willebrand disease (VWD)

A

Ristocetin cofactor assay of the patient’s plasma can measure the capacity of vWF to agglutinate platelets and is used in the diagnosis of VWD

Partial thromboplastin time will be prolonged due to decreased factor VIII

57
Q

Epididymitis

A

Testicular swelling in sexually active males

Positive Prehn’s sign (relief of pain with testicular elevation)

Normal lie and cremasteric reflex

58
Q

Organisms that cause Epididymitis

A

Younger than 35 years: Chlamydia trachomatis Doxycycline AND IM ceftriaxone

Older than 35 years Escherichia coli Levofloxacin OR ofloxacin

59
Q

Hemophilia A

A

X-linked recessive disorder
Factor VIII deficiency

Intrinsic pathway of the coagulation cascade will be dysfunctional (PTT increased)

Everything else normal

60
Q

community-acquired pneumonia (CAP) treatment

A

Combination therapy and usually consists of ceftriaxone plus doxycycline or azithromycin

61
Q

Osteitis fibrosa cystica

A

Skeletal disorder caused by a surplus of parathyroid hormone (PTH) from overactive parathyroid gland

Can cause lytic lesions on bones

looks like brown tumor with gaint cells

62
Q

Acute Promyelocytic Leukemia

A

Subclass of AML
• Arrow pointing to classic Auer rod
• Translocation t(15;17)
• Associated with disseminated intravascular coagulation
• Treatment: Emergent all-trans retinoic acid

63
Q

Burkitt Lymphoma

A

• Classic “starry-sky” appearance with sheets
of atypical lymphoid cells (sky) with evenly
dispersed normal macrophages (stars)
• Translocation t(8;14) c-myc
• Jaw or abdomen tumors
• Aggressive non-Hodgkin lymphoma

64
Q

Hairy Cell Leukemia

A
  • Rare B cell lymphoma (< 2%)
  • Tartrate-resistant acid phosphatase stain positive
  • Dry bone marrow aspiration
  • Hair-like protrusions from cell
65
Q

Hodgkin Lymphoma

A

• Reed Sternberg cells, a giant cell with a
multinucleated or bilobed nucleus resembling
owl’s eyes
• Pain with alcohol ingestion
• Cyclical fevers

66
Q

Chronic Lymphocytic Leukemia

A

• Smudge cell lacking identifiable cytoplasm
or nuclear structure
• Indolent leukemia, often found incidentally
• Affects older individuals

67
Q

laboratory findings for von Willebrand disease

A

Increased PTT

Normal PT

Normal Platelet count

68
Q

laboratory findings for Hemophilia A`

A

Increased PTT

Normal PT

Normal platelet count

69
Q

laboratory findings for DIC

A

Increased PT, PTT

Increased fibrin split products,

Decreased Platelet count and fibrinogen

70
Q

Causes of Death

A

Less than 1- Congenital problems

Adolescents- 1) Accidents. 2) suicide

Adults 45-65 1) Cancer 2) Heart Dz

Adults 65 and up 1) Heart dz 2) Cancer

71
Q

Waldenstrom macroglobulinemia

A

IgM

Hyperviscosity syndrome that typically manifests as lethargy, weight loss, Reynaud syndrome, and blurry vision

Tx: Plasmapheresis

72
Q

Multiple myeloma

A

CRAB (calcium [elevated)], renal failure, anemia, bone lesions) and IgG/IgA

73
Q

Monoclonal gammopathy of undetermined significance

A

IgG/IgA without CRAB