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Step 2 CK > Hematology > Flashcards

Hematology Flashcards

1
Q

What genetic type of alpha thal has high reticulocytes?

A

3 gene deletion

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2
Q

when 4 genes are deleted in alpha thal what can you expect?

A

gamma-4 tetrads or hgb bart; CHF causes death in utero

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3
Q

iron chelators needed for excessive transfusion in patients with Beta thalassemia?

A

Oral iron chelators: deferiprone and deferasirox

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4
Q

Goats milk, psoriasis, phenytoin and sulfa drugs and methotrexate use can all cause a deficiency in?

A

Folate

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5
Q

What is a complication of B12 or Folate replacement? why?

A

Hypokalemia: extremely rapid cell production in bone marrow causes hypokalemia.

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6
Q

Why might pancreatic enzyme deficiency cause B12 deficiency?

A

Pancreatic enzymes are needed to remove B12 from R-protein so that it can bind intrinsic factor and be reabsorbed

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7
Q

Genetic cause of sickle cell?

A

point mutation at position 6 of beta globin chain. valine replaces glutamic acid

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8
Q

2 things that lower mortality in sickle cell disease?

A

hydroxyurea in prevention

antibiotics with fever

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9
Q

Morulae are?

A

seen inside of neutrophils in Ehrlichia infection

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10
Q

how does hydroxyurea benefit sickle cell patients?

A

reduces recurrence of sickle cell crises by increasing hemoglobin F

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11
Q

When is exchange transfusion indicated for sickle cell disease?

A

Acute chest syndrome, priapism, stroke, retinal infarct

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12
Q

First clue to parvovirus in patient with sickle cell?

A

sudden drop in reticulocyte count

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13
Q

What is the first test you should order in a patient with sickle cell with a sudden drop in Hct?

A

Reticulocyte count (usually retic count is elevated in sickle cell due to chronic hemolysis)

Suspect parvo

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14
Q

most accurate test for parvo infection? best initial therapy?

A

PCR for DNA

IV immunoglobulin

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15
Q

2 benign manifestations of sickle cell trait?

A

isothenuria (inability concentrate urine)

hematuria

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16
Q

Most likely diagnosis for recurrent episodes of hemolysis, intermittent jaundice, splenomegaly, family history of anemia/hemolysis, bili gallstones

A

hereditary spherocytosis

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17
Q

MCHC is elevated in?

A

hereditary spherocytosis

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18
Q

Treatment for hereditary spherocytosis?

A

splenectomy

folic acid

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19
Q

What 2 diseases are associated with spherocytes?

A

HS and autoimmune hemolysis

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20
Q

Why doesn’t a smear show fragmented cells in autoimmune hemolysis?

A

red cell destruction occurs in spleen or liver. not blood vessel

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21
Q

best initial therapy for autoimmune hemolysis?

A

glucocorticoids

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22
Q

recurrent episodes of autoimmune hemolysis respond to? severe, acute hemolysis that doesn’t respond to prednisone should be treated with?

A

splenectomy

IVIG

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23
Q

Alternate treatments for autoimmune hemolytic anemia to decrease steroid use if splenectomy doesn’t work?

A

cyclophosphamide, cyclosporine, azathioprine, mycophenolate

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24
Q

Cold agglutinin anemia occurs in association with what diseases?

A

EBV, Waldenstroms, Mycoplasma Pneumo

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25
Q

Treatment for cold agglutinin autoimmune hemolytic anemia?

A

stay warm! rituximab! plasmapharesis. cyclophosphamide, clyclosporine

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26
Q

most common wrong answer for treatment of cold agglutinin? what else doesn’t work to treat?

A

Prednisone (doesn’t work!)

splenectomy! (wrong)

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27
Q

Cryoglobulinemia is associated with?

A

Hep C, joint pain, glomerulonephritis

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28
Q

Best initial test for G6PD? most accurate test for G6PD after 1-2 weeks?

A

initial: heinz bodies and bite cells on smear

most accurate: G6PD level 1-2 weeks later

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29
Q

HUS and TTP are both caused by deficiency in?

A

ADAMTS13

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30
Q

What will worsen the disease in patients with TTP and HUS?

A

platelet transfusion

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31
Q

Treatment for TTP and HUS?

severe cases?

A

Steroids

Plasmapharesis

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32
Q

Deficiency in compliment regulatory proteins CD55 and 59?

A

PND -> leads to overactivation of complement system

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33
Q

most common causes of death in PND?

A

thrombosis of mesenteric or hepatic veins

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34
Q

episodic dark urine, pancytopenia, iron deficiency, clots in weird places?

A

PND

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35
Q

best test for PND diagnosis?

A

flow cytometry /decreased CD 55-59

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36
Q

method of cure for PND

A

bone marrow transplant

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37
Q

Treatment of PND?

A

Prednisone- initial therapy

Eculizumab- inactivates C5

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38
Q

aplastic anemia is?

A

pancytopenia of unclear etiology. autoimmune in which T cells attack patients own marrow.

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39
Q

Treatment for aplastic anemia

A

If > 50 or no matched donor: cyclosporine: inhibit T cells and antithymocyte globulin (ATG)
If < 50 and matched donor: allogeneic transplant of bone marrow

40
Q

Target hct for polycythemia vera?

A

< 45%

41
Q

Treatment for P. vera

A
  1. phlebotomy and aspirin prevent thrombosis
  2. hydroxyurea
  3. allopurinol
  4. antihistamine
42
Q

MOA of ruxolitinib

A

inhibits JAK2

can be used in P. vera
essential thrombocytopenia
myelofibrosis

43
Q

Platelet count in essential thrombocytosis is often >?

A

1 million

44
Q

best initial therapy for essential thrombocytosis if patients is > 60 and has thromboses?

A

hydroxyurea

45
Q

tear drop cells and nucleated RBCs on peripheral smear?

A

Myelofibrosis

46
Q

Thalidomide and lenalidomide are useful in myelofibrosis treatment because?

A

TNF inhibitors. increase bone marrow production

47
Q

A history of myelodysplastic syndrome might suggest?

A

acute leukemia

48
Q

Acute promyelocytic leukemia is associated with what condition?

A

DIC

49
Q

Myeloperoxidase is characteristic of?

A

acute myelocytic leukemia

50
Q

Rasburicase MOA?

A

prevents tumor lysis related rise in uric acid

51
Q

What therapy is important to add for ALL treatment?

A

Add intrathecal methotrexate!

52
Q

Auer rods = ?

A

AML

53
Q

Add ATRA for treatment with?

A

M3 (Promyelocytic leukemia)

54
Q

Most accurate test to diagnose CML?

A

BCR-ABL (done by PCR or FISH) on peripheral blood

55
Q

Blood smear findings in CML

A

persistently high WBC count all neutrophils. <5% blasts. increased basophils

56
Q

Most effective cure for CML?

A

BMT

57
Q

Best initial treatment for CML?

A

tyrosine kinase inhibitor

58
Q

CML has the greatest likelihood of all myeloproliferative disorders to transform into…?

A

Acute leukemia (blast crisis)

59
Q

A patient presents with symptomatic WBC count of 225,000. what is your first step in management?

A

Leukapharesis

60
Q

5q deletion is standard abnormality in? Patients with 5q have better or worse prognosis?

A

Myelodysplastic syndrome

Better prognosis

61
Q

5q deletion in MDS responds to what drug?

A

lenalidomide

62
Q

Severity of MDS is based on?

A

number of blast cells

63
Q

Pelger-Huet cells

A

bilobed nucleus. characteristic cells of MDS

64
Q

Azacitidine significance in MDS

A

decreases transfusion dependence and increases survival

65
Q

Richter phenomenon

A

conversion of CLL into a high-grade lymphoma

66
Q

Smudge cell

A

lab artifact where fragile nucleus is crushed by a cover slip (seen in CLL)

67
Q

Prophylaxis for what bug is indicated in CLL?

A

PCP

68
Q

Treat refractory cases of CLL with?

A

cyclophasphamide

69
Q

Mild cases of CLL in the elderly with?

A

chlorambucil

70
Q

Severe CLL infection treat with?

A

IVIG

71
Q

Fludarabine, cyclophosphamide, rituximab are best therapy for??

A

CLL

72
Q

Dry tap despite hypercellularity of bone marrow suggests?

A

Hairy cell leukemia (B-cells with filamentous projections

73
Q

Cladiribine or pentostatin are good treatments for?

A

Hairy cell leukemia

74
Q

best initial test to diagnose non hodgkins lymphoma?

A

excisional biopsy

75
Q

How often does NHL present in an advanced stage?

A

80% - 90% of the time

76
Q

For advanced disease of NHL treatment is with?

A

R-CHOP

77
Q

Hodgkin lymphoma centers around what part of body?

A

cervical area

78
Q

Which type of hodgkin has worst prognosis?

A

lymphocyte depleted

79
Q

which type of non-hodgkin has worst prognosis?

A

Burkitt and Immunoblastic

80
Q

Treatment for stage III or IV hodgkin ?

A

ABVD

81
Q

You cant use adriamycin if ejection fraction is less than?

A

50%

82
Q

What is the most useful test to perform to determine the dosing of chemotherapy in Hodgkin lymphoma?

A

MUGA or nuclear ventriculogram because Adriamycin (dano and doxorubicin are cardio toxic)

83
Q

Adverse effect of doxorubicin (adriamycin)

A

cardiotoxicity

84
Q

adverse effect of vincristine

A

peripheral neuropathy

85
Q

adverse effect of bleomycin

A

lung fibrosis

86
Q

cisplatin adverse effect

A

ototoxicity and nephrotoxicity

87
Q

cyclophosphamide adverse effect

A

hemorragic cystitis

88
Q

most common presentation of mulitple myeloma?

A

bone pain from pathologic fractures

89
Q

Reason for bone pain, breaks and hypercalcemia in multiple myeloma?

A

osteoclast activating factor is released

90
Q

most common causes of death in multiple myeloma

A

renal failure and infection

91
Q

greater than ____ % plasma cells defines myeloma

A

10 %

92
Q

Why does multiple myeloma have an decreased anion gap?

A

IgG is cationic. cations increase chloride and bicarb levels.

93
Q

Does urine dipstick detect jones protein?

A

no (need urine electrophoresis)

94
Q

Single most accurate test to diagnose multiple myeloma?

A

greater than 10% plasma cells on bone marrow biopsy (most common wrong answer is SPEP).

95
Q

Why isnt SPEP the best test for MM?

A

99% of people with M spike do not have multiple myeloma. they have MGUS

96
Q

lethargy, blurry vision with vertigo, engorged blood vessels in eye, mucosal bleeding, raynauds is a common presentation for?

A

Waldenstrom Macroglobulinemia

97
Q

Best therapy for Waldenstroms

A

plasmapharesis

Step 2 CK (26 decks)

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