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Flashcards in Hematology Deck (137):
1

Best initial test when concern for anemia

CBC with peripheral smear

2

What are supplementary tests to order on CCS when concerned about anemia?

Reticulocyte count, Iron labs, TSH and T4, haptoglobin, bilirubin, LDH, B12/folate

3

What type of anemia is caused by blood loss?

Iron deficiency

4

What are iron labs in iron deficiency anemia?

Iron: Low
FERRITIN: LOW
TIBC: High
Transferrin saturation: Low
Elevated RDW

5

Most accurate test to dx IDA

Bone marrow biopsy (do not order on CCS)

6

Tx for IDA

Oral ferrous sulfate

7

Iron studies in anemia of chronic disease

Iron: Low
FERRITIN: HIGH
TIBC: Low
Transferrin saturation: Low or normal

8

Buzzword

Microcytic anemia with target cells

Thalassemia

9

Most accurate test for dx of thalassemia

Hemoglobin electrophoresis
(Beta form: elevated HgA2 and HgF)
(Alpha form: normal)

10

Is sideroblastic anemia microcytic or normocytic?
What are potential causes (3)?

Microcytic
Alcohol, Isoniazid, Lead exposure

11

Iron levels in sideroblastic anemia are ....

High

12

Most accurate test for dx of sideroblastic anemia

Prussian blue stain

13

Alpha thalassemia is most accurately diagnosed by ...

DNA sequencing

14

What is one of the only anemias with a high RDW and why?

IDA
As there is less iron the RBCs made are smaller and smaller which leads to large variation in size

15

Besides fatigue what else does B12 deficiency anemia present with?

Peripheral neuropathy but can be other neurologic problems like dementia

16

Buzzwords

Glossitis and diarrhea associated anemia

B12 deficiency

17

Buzzwords

Hypersegmented neutrophils and oval cells

Macrocytic anemia

18

What happens to reticulocyte count in B12 deficiency anemia?

Low
B12 deficiency causes destruction of cells just as they leave bone marrow which causes low reticulocyte count

19

What happens to homocysteine and methylmalonic acid levels in B12 and folate deficiency?

B12: homocysteine and methylmalonic acid levels rise
Folate: homocysteine levels rise

20

You've confirmed a low B12 and high methylmalonic acid. What is the next test you should run for etiology?

Antiparietal cell Abs and anti-intrinisic factor Abs to look for pernicious anemia

21

Hemolytic anemia labs

Indirect bili, reticulocyte count, LDH, haptoglobin, PBS

Indirect bilirubin: High
Reticulocyte count: High
LDH: High
Haptoglobin: Low
Spherocytes on PBS

22

What should you order for the PE on CCS for sickle cell anemia?

Complete physical exam

23

What are best initial therapies to offer a patient presenting with sickle cell anemia pain?

Oxygen, pain meds, fluid hydration

24

Is fever in sickle cell anemia a emergency?
What medication should you give?

Yes
Levofloxacin, moxifloxacin, or ceftriaxone

25

What should be repleted in all sickle cell patients?

Folate

26

Most accurate test for parvovirus B19 dx in sickle cell patients

Parvovirus DNA PCR

27

Parvovirus infection in sickle cell is best treated how?

Transfusions and IVIG treatment

28

At discharge what should sickle cell patients be discharged on?

Folate
Hydroxyurea
Pneumococcal vaccination

29

What is the only manifestation of hemoglobin SC disease?

Renal: hematuria, UTI, isosthenuria (inability to concentrate urine)

30

Most accurate diagnostic test for autoimmune hemolysis

Coomb's test

31

Best initial therapy for autoimmune hemolysis

Steroids (prednisone)

32

If there are recurrent episodes of hemolysis in autoimmune hemolysis then what is the most effective therapy?

Splenectomy

33

Are 'warm Abs' IgM or IgG?

IgG

*Only IgG abs respond to steroids and splenectomy

34

Buzzword

Mycoplasma or EBV associated anemia

Cold-induced hemolysis

35

What will be the result of a standard Coomb's test in cold-induced hemolysis? What test will be positive?

Negative

Complement test will be positive

36

How is cold-induced hemolysis treated?

Rituximab

*No response to steroids, splenectomy, or IVIG

37

Is cold-induced hemolysis IgG or IgM?

IgM

38

What is mode of inheritance of G6PD deficiency?

X-linked recessive

39

What are common triggers of G6PD deficiency anemia?

Infection
Oxidizing drugs (e.g. sulfas, primaquine, dapsone)
Fava beans

40

Buzzwords

Heinz bodies and bite cells are seen in this anemia

G6PD deficiency

41

What is the most accurate test for dx of G6PD deficiency and when should it be ran?

G6PD level but should be done 2 months after the episode. If done too early it will be normal bc the most deficient cells will have been destroyed

42

Recurrent episodes of hemolysis, splenomegaly, bilirubin gallstones, and a high MCHC are signs of which anemia cause?

Hereditary spherocytosis

43

What is the most accurate diagnostic test for hereditary spherocytosis?

Osmotic fragility test

44

Tx of hereditary spherocytosis

Splenectomy

45

Buzzword

E. Coli 0157:H7

Hemolytic-Uremic Syndrome

46

Ticlodipine can predispose to what macroangiopathic hemolytic anemia?

Thrombotic thrombocytopenic purpura (TTP)

47

Findings in HUS

Intravascular hemolysis
Elevated BUN and creatinine
Thrombocytopenia

48

Findings in TTP (5)

Intravasacular hemolysis
Elevated BUN and creatinine
Thrombocytopenia
Fever
Neurological abnormalities

49

ADAMTS-13 level in TTP is ...

Low

50

Should you transfuse platelets in a patient with TTP or HUS?

No

51

Severe cases of TTP or HUS are treated with what?

Plasmapharesis

52

Patient presents with pancytopenia and dark urine only in am. Dx?

Paroxysmal nocturnal hemoglobinuria

53

What is most common cause of death in paroxysmal nocturnal hemoglobinuria?

Large vessel thrombosis

54

Most accurate test for diagnosis of PNH

CD55 or CD59 Ab (aka decay accelerating factor)

55

Best initial tx of PNH

Steroids

*Transfusion-dependent patients with severe disease are treated with eculizumab

56

What are some drugs which may cause methemoglobinemia?

Nitroglycerin, amyl nitrate, nitroprusside dapsone, -caines

57

What may cause SOB with clear lungs on exam, normal CXR, and brown blood? What is tx?

Methemoglobinemia
Methylene blue

58

A patient receiving blood transfusion and becoming anaphylactic with normal LDH and bilirubin is caused by ...

IgA deficiency

59

Delayed jaundice in a patient receiving a blood transfusion is caused by ...

Minor blood grouping incompatability

60

Buzzwords

Auer rods associated with

AML

61

Buzzwords

DIC associated leukemia

M3, acute promyelocytic leukemia

62

Best initial test in diagnosis of acute leukemia

PBS showing blasts

63

What is the most important prognostic indicator in acute leukemia which may be an indication for bone marrow transplantation in addition to chemo?

Cytogenetic abnormalities suggesting high rate of relapse

64

Chemo agents used in acute leukemia are?
What is added in M3 (acute promyelocytic) leukemia?
What is added for ALL?

Idarubicin (or daunorubicin) and cytosine arabinoside
In M3 add all-trans retinoic acid
In ALL add intrathecal MTX

65

In patients with extremely high WBC count in leukemia what is the recommended therapy?

Leukapheresis and hydroxyurea to reduce WBC count

66

What presents with pancytopenia, elevated MCV, low reticulocytes, and macroovalocytes?

Myelodysplasia

67

Are there more or less blasts in myelodysplasia compared to leukemia?

Less. Myelodysplasia is like a preleukemia syndrome which is slower and may ultimately progress to leukemia

68

Buzzword

Pelger-Huet cells association

Myelodysplasia

*The cell is a neutrophil with two lobes

69

What is the most common cause of death in patients with myelodysplasia?

Infection or bleeding

70

Tx of myelodysplasia

Supportive with transfusions as needed

*Azacitadine (increases survival), decitabine, and lenalidomide

71

Why do patients have early satiety in CML and CLL?

Splenomegaly may compress stomach

72

What happens to the following values in CML?

Leukocyte alkaline phosphatase (LAP)
Neutrophils

LAP decreases
Neutrophils increase

73

Elevated LAP score indicates ...

Leukomoid reaction (reactive increase in WBC)

74

Buzzword

Philadelphia chromosome

bcr/abl in CML responding to Gleevac (imatinib)

75

Best initial therapy CML

Imatinib

*BM transplantation cures CML but is never the best initial test

76

Best initial test for CLL

PBS showing lymphocyte smudge cells

77

Tx for CLL

Stage based. Early forms of disease do not need treatment.

Advanced stage disease: fludarabine (prolongs survival), rituximab, and cyclophosphamide

78

Should you treat elevated WBC count (asymptomatic) in patients with CLL?

No

79

If fludarabine fails in CLLthen what med is used?

Alemtuzumab (anti-CD52 agent)

80

Smear showing hairy cells and immunophenotyping are the most accurate tests/findings for what disease?

Hairy cell leukemia

81

Best initial therapy in hairy cell leukemia

Cladribine (2-CDA)

82

How can myelofibrosis and hairy cell leukemia be distinguished?

Hairy cell leukemia has elevated TRAP
Normal TRAP in myelofibrosis

83

Buzzword

Tear drop cells

Myelofibrosis

84

What mutation is seen in myelofibrosis?

JAK2

*Ruxolitinib inhibits JAK2 and is occasionally used

85

Best initial therapy for myelofibrosis

Lenalidomide or thalidomide

86

Headache, blurred vision, dizziness, and pruritis may be seen in what disorder?

Polycythemia vera

87

MCV in polycythemia vera

Low

88

JAK2, normal O2 sat, and low MCV are seen in ...

Polycythemia vera

89

Best initial therapy for polycythemia vera

Phlebotomy

90

Meds used for polycythemia vera and essential thrombocythemia

Hydroxyurea (lowers cell count)
Aspirin
Anagrelide (if thrombocythemia)

91

What is erythromelalgia?

Pain in hands and feet seen in erythrocythemia from high platelet count

92

What are most common causes of death of multiple myeloma?

Renal failure and infection

93

Elevated levels of monoclonal IgG antibody on SPEP may suggest what disease?

Multiple myeloma

94

Buzzwords

Bence-Jones protein on UPEP

Multiple myeloma

95

How will a peeripheral smear appear in multiple myeloma?

Rouleaux formation from RBC clumping in chains

96

What is a prognostic indicator in multiple myeloma?

Beta-2 microglobulin

97

What medicine reverses the renal dysfunction in multiple myeloma?

Bortezomib

98

B12 and LAP levels are changed how in polycythemia vera?

Both elevated

99

What is the most specific test for diagnosis of multiple myeloma?

Bone marrow biopsy

100

Tx of multiple myeloma

Melphalan and steroids
Thalidomide, bortezomib, and lenalidomide may be added

101

Patients that are relatively young (

Autologous stem cell BM transplantation

102

Patients with hypercalcemia in multiple myeloma should be managed how?

Hydration and diuresis

103

Asymptomatic elevation of IgG on SPEP

Monoclonal gammopathy of unknown significance

104

What are the best initial tests in Waldenstrom's Macroglobulinemia?

Serum viscosity test
SPEP with elevated IgM

105

Best initial therapy for symptomatic patients with Waldenstrom's

Plasmapharesis
(additional tx are rituximab fludarabine, chlorambucil, or rituximab)

106

Chronic Hep B and C can cause ...

Aplastic anemia

107

What are therapy options for patients with aplastic anemia?

If young and a match BM transplant. If not either then antithymocyte globulin and cyclsporine

108

How are Hodgkin's disease and Non-Hodgkins's disease different in terms of disease localization?

Hodgkin's: Starts centrally, near cervical region and spreads out from center
Non-Hodgkin's: Widespread

109

What is the best initial test for diagnosing Hodgkin's and NHL?

Excisional lymph node biopsy

110

Histologically how do Hodgkin's and NHL differ

Hodgkin's has Reed-Sternberg cells (look like owls)

111

Describe the staging scheme for lymphoma

Stage I: Single lymph node group
Stage II: Two lymph node groups on one side of diaphragm
Stage III: Lymph node groups on both sides of diaphragm
Stage IV: Widespread disease

112

What other tests are helpful in staging lymphoma?

CXR
BM biopsy
CT scan with contrast

113

How are the different stages of lymphoma treated in general terms (e.g. radiation, chemo, etc)?

Stages I and II: Radiotherapy and chemo
Stage III and IV: Chemo

114

Tx for Hodgkin's Disease

ADVB
Adriamycin, bleomycin, vinblastine, dacarbazine

115

Tx for NHL

CHOP
Cyclophosphamide
Hydroxyadriamycin
Oncovin (vincristine)
Prednisone

If anti-CD20 positive then add Rituximab

116

Von Willenbrand's Disease is caused by what abnormality? What happens to platelet and clotting times?

Platelet dysfunction from lack of vWF
Platelet counts are normal
PTT may be elevated because vWF stabilizes factor VIII

117

What are the most accurate tests for diagnosing vWD?

Ristcetin cofactor assay (can tell if vWF is working even if normal)
vWF level

118

What is first-line treatment for vWD?

Desmopression (DDAVP)

119

If DDAVP does not work for vWD then what is another treatment option?

Factor VIII replacement (contains both vWD and factor VIII)

120

Antibodies to what receptor cause ITP?

Antibodies to glycoprotein IIb/IIIa receptor

121

Peripheral smear demonstrating large platelets and BM showing megakaryocytes may be indicative of what disorder?

ITP

122

If ITP is suspected what is the best next step?

Provide prednisone. It is a diagnosis of exclusion and time should not be spent trying to diagnosis

123

In severe ITP presenting low platelet count (e.g.

Provide IVIG

124

What do romiplostim and eltrombopag do?

They're thrombopoietin analogs which directly stimulate megakaryocytes and can be used to treat chronic ITP

125

Normal ristocetin and vWF levels in a patient with platelet-type bleeding and kidney failure indicates what problem?

Uremia-induced platelet dysfunction

126

What is specifically disrupted in platelets from patients with uremia-induced platelet dysfunction?

Their ability to degranulate

127

Tx of uremia-induced platelet dysfunction

DDAVP, dialysis, estrogen

128

What is treatment for factor XII deficiency?

No treamtent needed

129

What is treatment for factor XI deficiency?

Fresh frozen plasma

130

Does thrombosis in HIT occur more in veins or arteries?

Veins

131

Best initial diagnostic tests for HIT

Platelet factor 4 Abs or heparin-induced platelet antibodies

132

Buzzword

Russel viper venom test

Most accurate test for lupus anticoagulant

133

What are clotting times in antiphospholipid syndrome and other unique lab results?

Elevated aPTT
Normal PT
False positive VDRL

134

Tx of APAS

Heparin followed by warfarin

135

How does protein C deficiency present when starting anticoagulation?

Skin necrosis when patients go on warfarin
Venous thrombosis

136

What is treatment of protein c deficiency?

Heparin followed by warfarin

137

What is MOA of heparin?

Inactivates thrombin and activated Xa