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Flashcards in MTB - Hematology Deck (125):
1

In older patient with anemia - what level of Hb is your goal?

Above 10

2

Iron studies in anemia of chronic disease

High ferritin
Low TIBC
Low Fe
Normal/low Fe sat

3

Electrophoresis in B thalassemia

Elevated HbA2 and HbF

4

Hb electrophoresis in A-thalassemia

Normal

5

What are potential causes of sideroblastic anemia?

Alcoholic
Lead exposure
Isoniazid

6

Most accurate diagnostic test for sideroblastic anemia

Prussian blue stain

7

Treatment of sideroblastic anemia

1. Remove toxin exposure
2. Pyridoxine replacement

8

Most accurate test for alpha thalassemia

DNA sequencing

9

Which anemia has increased RDW?

Iron deficiency only

10

Only microcytic anemia with high reticulocyte count

HbH - 3 alpha gene deletion

11

Anemia of chronic disease

Normal or increased amounts of iron and ferritin in storage but inability to process the iron into a usable form for Hb synthesis

12

EPO can be used to tx what anemia?

Anemia of chronic disease due to end stage renal disease

13

Three unique signs of vit B12 deficiency

Neuropathy (peripheral)
Glossitis (smooth tongue)
Diarrhea

14

Which drug can block vit B12 absorption?

Meteor in

15

Peripheral blood smear findings in megaloblastic anemia

Hypersegmented neutrophils (>4 lobes)
Oval cells

16

What other lab tests should you get in macrocytic anemia?

Bilirubin and LDH - will be increased
Reticulocyte count - will be decreased

17

Your suspect vit B12 deficiency in your patient with macrocytic anemia, but their b12 level is normal - what other test can help you distinguish between vitamin B12 and folate deficiency?

Methylmalonic acid level - will be elevated in B12 def

Homocysteine is elevated in both

18

You found low vitamin B12 levels and elevated methylmalonic acid levels in your patient - what's the next test to determine etiology of deficiency?

Anti-parietal cell abs or anti- intrinsic factor abs

19

Lab findings in hemolytic anemia

Elevated bilirubin, LDH and reticulocytes
Decreased haptoglobin

20

In addition to regular findings of hemolysis, intravascular hemolysis shows...

Schistocytes, helmet cells and fragmented cells on smear
Hemoglobinuria
Hemosiderin urea

21

A patient with sickle cell anemia presents with severe pain in the chest, back and thighs. What is your initial management?

Oxygen
IVF with normal saline
Pain meds

22

Patient with sickle cell crises, develops fever while on oxygen and hydration. What do you do?

Give abx: ceftriaxone, levofloxacin or moxifloxacin
Perform complete physical exam
Order blood cultures, urinalysis, reticulocyte count, CBC and CXR

23

In what cases would you give an exchange transfusion to a sickle cell pt

Visual disturbance
Pulmonary infarction
Priapism
Stroke

24

Sickle cell pt admitted for pain crisis has a drop in htc with rise in reticulocyte count over two days - what do you suspect?

Either folate deficiency or parvovirus infection
If pt is receiving replacement therapy, it is likely parvo

25

Dx and Tx of parvovirus in sickle cell pt

PCR for DNA of parvovirus
Tx, transfusion, IVIG

26

What meds do you discharge a sickle patient?

Folate replacement
Pneumococcal vaccine
Hydroxyurea - if >4 episodes per year

27

CF in sickle cell trait

Renal problems
- hematuria
- isostenuria (prone to dehydration)
- urinary tract infections

28

Causes of autoimmune hemolysis

Other autoimmune conditions:SLE, RA
CLL
MEDS: penicillin, alpha-methyl dopa, quinine, sulfa drugs

29

Most accurate diagnostic test in autoimmune hemolysis

Coombs test

30

Best initial therapy in autoimmune hemolysis

Steroids (prednisone)
If recurrent - splenectomy
Only IgG abs respond to these treatments

31

If a case describes severe hemolysis that does not respond to prednisone or repeated transfusions - what can you do?

Give IVIG

32

Features of cold induced hemolysis

Mycoplasma or EBV in history
Coombs negative
Complement positive
No response to steroids, transfusion or IVIG

33

Tx. Cold hemolysis

Rituximab

34

Which anemia is X-linked?

G6PD Deficiency

35

Causes of acute oxidant stress in g6pd deficiency

Infection
Sulfa drug, primaquine, dapsone
Fava beans

36

Best initial test in g6pd deficiency

Heinz body test - Heinz bodies and bite cells on smear

37

Most accurate test for G6PD deficiency

G6pd level - wait two weeks after acute episode to measure level (will be normal right after episode )

38

Tx. G6PD deficiency

No tx - avoid oxidant stress

39

CF in hereditary spherocytosis

Recurrent episodes of hemolysis
Splenomegaly
Bilirubin gallstones
Elevated MCHC

40

Causes of HUS / TTP

E.coli O157:H7
Ticlopidine

41

HUS triad

Hemolysis
Renal failure
Thrombocytopenia

42

TTP

Hemolysis
Renal failure
Thrombocytopenia
Fever
Neurological changes

43

Tx. HUS / TTP

Plasmapheresis

44

Patient is presenting with pancytopenia and recurrent episodes if dark urine - dx? Test?

Dx: PNH
Test - c55 and c59 ab level

45

Tx. PNH

Steroids
If transfusion dep. try eculizumab

46

What drugs can cause methemoglobinemia?

Nitroglycerin
Amyl nitrate
Nitroprusside
Dapsone
Anesthetics ending in a sine

47

Tx. Methemoglobinemia

Methylene blue

48

Acute symptoms of hemolysis while transfusion is occuring

ABO incompatibility

49

Acute SOB shortly after transfusion that goes away on its own

Transfusion assoc. Acute lung injury
- donor abs against WBCs
- no tx

50

What transfusion reaction presents with delayed jaundice and no other symptoms?

Minor blood group incompatibility
No specific therapy

51

Best initial test if you suspect leukemia

Peripheral smear - will show blasts

52

Most impt prognostic test in leukemia

Cytogenetics ex. Karyotypic analysis

53

Tx. AML

Chemotherapy with idarubicin and cytosine arabinoside

54

What extra tx. Is given in M3 AML

All trans retinoic acid

55

Additional therapy for ALL

Intrathecal methotrexate

56

Leukostasis as the initial presentation of acute leukemia

Slushing of blood in blood vessels of eyes, brain and lungs when WBC is > 100,000.

57

Tx. Leukostasis

Leukaphoresis
Hydroxyurea

58

Pelger-Huet cell

Neutrophil with two lobes seen in myelodysplasia

59

Pt presents with pancytopenia, elevated MCV, low retic count and macroovulocytes with Pelger Huet cells. Dx?

Myelodysplasia

60

Tx. Myelodysplasia

Supportive, transfusions as needed
Azacitadine - specific therapy

61

Lenalidomide

Drug approved for 5q minus syndrome form of myelodysplasia

62

Pt presents with elevated WBC predominantly neutrophils and splenomegaly - dx?

CML

63

Best initial test in CML

LAP score
- elevated neutrophils but LOW LAP

64

Most accurate test for cml

Philadelphia xm

65

Tx. CML

Imatinib (also can use: dasatinib or nilotinib)
BMT only if no response to medical therapy

66

55 year old patient presents with elevated WBC on routine CBC. It is predominantly lymphocytes. Dx? Best initial test?

Dx. CLL
TEST: peripheral smear will show smudge cells

67

Tx. CLL

Based on stage
Stage 0-1: elevated cells, enlarged LN - no therapy
Stage 2-4: splenomegaly, anemia, low platelets - fludarabine + rituximab

68

Alemtuzumab

Anti CD52 AB used in tx of CLL

69

55 yo pt presents with pancytopenia and massive splenomegaly. dx? Test?

Dx. Hairy cell leukemia
Test: trap stain, peripheral smear shows hairy cells

70

Tx. Hairy cell leukemia

Cladribine (2-CDA)

71

55 yo patient presents with pancytopenia and splenomegaly. TRAP stain is negative. Smear shows tear drop cells. Dx? Tx?

Myelofibrosis
Tx. BMT
If BMT is not possible, best initial therapy is Lenalidomide or thalidomide

72

Lab findings in polycythemia Vera

Elevated Htc, WBC and platelets
Decreased MCV and EPO

73

A patient has high hematocrit on CBC, what test do you order next?

Arterial blood gas
- want to rule out hypoxia as a cause of erythrocytes is
EPO level
Jak2 mutation

74

Tx. Polycythemia Vera

Phlebotomy
Hydroxyurea - can decrease red count
Daily aspirin

75

Tx. Multiple myeloma

Melphalan and steroids

76

Most effective therapy for multiple myeloma

Autologous BMT

77

Prognostic indicator in multiple myeloma

Beta 2 micro globulin level

78

MGUS

Asymptomatic elevation of IgG in elderly person with total elevated protein. No treatment

79

Pt presents with blurry vision, confusion, headache, enlarged LN and spleen. IgM levels are elevated - dx

Waldenstrom macroblobulinemia

80

Best initial test in Waldenstrom macroglobulinemia

Serum viscosity test or SPEP

81

Best initial therapy for Waldenstrom macroglobulinemia

Plasmapheresis

82

Best initial test for Hodgkins and NHL

Excisional lymph node biopsy

83

Chemo tx for hodgkins

Adriamycin
Bleomycin
Vinblastine
Dacarbazine

84

Chemotherapy for NHL

Chop regimen
Cyclophosphamide
Hydroxyadriamycin
Oncovin (vincristine)
Prednisone

85

Pt presents with superficial bleeding from skin and mucosal surfaces. Platelet count is normal. Bleeding gets worse every time they take aspirin. Dx?

Von willebrand dz

86

Most accurate test for Von willebrand dz

Ristocetin cofactor assay and vWF level

87

First line tx of Von willebrand dz

Desmopressin or DDAvp
- releases subendothelial stores of vWF and factor VIII

88

Sudden appearance of rash and or bleeding in otherwise healthy child

Consider ITP

89

What antibodies are present in ITP

Glycoproteins IIB/IIIa receptor abs

90

Tx. Mild ITP (mild bleeding with platelets <50,000)

Prednisone

91

Tx. ITP with platelet count <20,000 and serious bleeding (intracranial hemorrhage)

IVIG or Rho gam

92

What drugs can be used in treatment of ITP if no response to splenectomy and still having recurrent episodes?

Romiplostim
Eltrombopag

93

Tx. Uremia induced platelet dysfunction

Desmopressin

94

Prolonged aPTT and bleeding that does not correct with a mixing study

Factor VIII antibody - MCC

95

Pt who was given heparin three days ago for DVT prophylaxis comes in a new thrombosis - what should u be considering?

Heparin induced thrombocytopenia - drop in platelets few days after heparin treatment; thrombosis is most common clinical manifestation

96

Best initial diagnostic test for HIT

Platelet factor 4 abs
Heparin induced anti platelet abs

97

CCS - what physical exam stuff do you order for suspected anemia?

General appearance, cv, chest, extremities, HEENT

98

Best initial therapy for HIT

Stop heparin
Give direct thrombin inhibitor (argatroban, lepirudin)

99

Tx. HIV associated thrombocytopenia

Zidovudine
- min 600 mg/day
Corticosteroids will work but will recur after stopping them and can increase risk of opportunistic infections

100

If an HIV positive patient adheres to a 3 drug HARRT regimen - when will their viral load decrease to < 50 copies/ml?

16-24 weeks
- measure viral load at 4 weeks, 8-12 weeks and then remeasure every 6-8 weeks thereafter

101

tx. of inoperable head and neck cancer

combination chemo-radiotherapy

102

Type 1 HIT

w/in 2d of initiation of heparin therapy; platelet count returns to normal with d/c of therapy

103

Type 2 HIT

abs against heparin platelet factor 4 which causes platelet activation and aggregation leading to thrombocytopenia and platelet rich clots; usually 4-10 days after initiation of heparin therapy and can lead to arterial or venous thrombosis

104

warfarin induced skin necrosis

high dose warfarin induces a transient hypercoagulable state by causing rapid reduction in protein C levels on the first day of therapy; lesions on breasts, trunk and extremities. No thrombocytopenia

105

heparin induced skin necrosis

complication of unfractionated heparin; involves areas rich in fat

106

Tx. in HIT

discontinuation of all heparin products and warfarin and start anticoagulation with direct thrombin inhibitors (lepirudin, argatroban)

107

limitations of lepirudin and argatroban

lepirudin - used in caution in renal insuff pts
argatroban - use in caution with hepatic dysfunction

108

MCC of anemia in pts with ESRD

EPO deficiency

109

absolute iron deficiency

< 100 ng/ml

110

iron supplementation in pts with ESRD is recommended when

when transferrin sat < 30% and ferritin < 500ng/ml
- iron supp. should be IV for pts receiving dialysis for ESRD

111

pt with low Hb despite being on EPO, ferritin > 500 - next step/

increase dose of EPO

112

tx. of anemia of chronic disease in patients with RA

anti-TNF agents, i.e. infliximab

113

next step in eval of normocytic normochromic anemia

reticulocyte count
- to r/o hemolytic causes

114

major problem leading to difficulty finding cross-matched blood in patients with a history of multiple transfusions

alloantibodies - most commonly, E, L and K

115

indications for irradiated RBCs

BMT recipients
immunodeficiency pts
blood components donated by first or second degree relatives

116

indications for leukoreduced RBCs

chronically transfused pts
CMV seronegative at risk pts (HIV, transplants)
potential transplant recipients
previous febrile non hemolytic transfusion reaction

117

indications for washed RBCs

IgA deficiency
complement dep. autoimmune hemolytic anemia
continued allergic reactions with transfusion despite antihistamine treatment

118

initial diagnostic test for pernicious anemia

anti-IF ab testing

119

findings on biopsy in pernicious anemia

absent rugae in fundus and body--> autoimmune metaplastic atrophic gastritis

120

EPO levels in polycythemia vera

normal or low

121

polycythemia + high EPO levels

secondary causes
- consider getting nocturnal oximetry and carboxyhemoglobin

122

tx. multiple brain mets

whole brain irradiation
- can improve survival 3-6 months

123

screening while on tamoxifen

no additional screening needed
- annual gyn exam with complete history and routine pap smears

124

1 unit of platelets should increase platelet count by how much

5000 platelets
- measure post transfusion count 10-60 minutes following transfusion

125

platelet refractoriness

absolute platelet count increment of less than or equal to 2000 / unit of platelets given
- MCC - alloimmunizatin (ab to HLA class 1 antigen on platelets)