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Step 3 > Hematology > Flashcards

Hematology Flashcards

1
Q

Best initial test when concern for anemia

A

CBC with peripheral smear

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2
Q

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

A

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

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3
Q

What type of anemia is caused by blood loss?

A

Iron deficiency

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4
Q

What are iron labs in iron deficiency anemia?

A 
Iron: Low
FERRITIN: LOW
TIBC: High
Transferrin saturation: Low 
Elevated RDW
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5
Q

Most accurate test to dx IDA

A

Bone marrow biopsy (do not order on CCS)

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6
Q

Tx for IDA

A

Oral ferrous sulfate

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7
Q

Iron studies in anemia of chronic disease

A

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

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8
Q

Buzzword

Microcytic anemia with target cells

A

Thalassemia

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9
Q

Most accurate test for dx of thalassemia

A

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

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10
Q

Is sideroblastic anemia microcytic or normocytic?

What are potential causes (3)?

A

Microcytic

Alcohol, Isoniazid, Lead exposure

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11
Q

Iron levels in sideroblastic anemia are ….

A

High

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12
Q

Most accurate test for dx of sideroblastic anemia

A

Prussian blue stain

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13
Q

Alpha thalassemia is most accurately diagnosed by …

A

DNA sequencing

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14
Q

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

A

IDA

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

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15
Q

Besides fatigue what else does B12 deficiency anemia present with?

A

Peripheral neuropathy but can be other neurologic problems like dementia

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16
Q

Buzzwords

Glossitis and diarrhea associated anemia

A

B12 deficiency

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17
Q

Buzzwords

Hypersegmented neutrophils and oval cells

A

Macrocytic anemia

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18
Q

What happens to reticulocyte count in B12 deficiency anemia?

A

Low

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

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19
Q

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

A

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

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20
Q

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

A

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

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21
Q

Hemolytic anemia labs

Indirect bili, reticulocyte count, LDH, haptoglobin, PBS

A 
Indirect bilirubin: High
Reticulocyte count: High
LDH: High
Haptoglobin: Low
Spherocytes on PBS
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22
Q

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

A

Complete physical exam

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23
Q

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

A

Oxygen, pain meds, fluid hydration

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24
Q

Is fever in sickle cell anemia a emergency?

What medication should you give?

A

Yes

Levofloxacin, moxifloxacin, or ceftriaxone

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25
Q

What should be repleted in all sickle cell patients?

A

Folate

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26
Q

Most accurate test for parvovirus B19 dx in sickle cell patients

A

Parvovirus DNA PCR

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27
Q

Parvovirus infection in sickle cell is best treated how?

A

Transfusions and IVIG treatment

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28
Q

At discharge what should sickle cell patients be discharged on?

A

Folate
Hydroxyurea
Pneumococcal vaccination

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29
Q

What is the only manifestation of hemoglobin SC disease?

A

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

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30
Q

Most accurate diagnostic test for autoimmune hemolysis

A

Coomb’s test

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31
Q

Best initial therapy for autoimmune hemolysis

A

Steroids (prednisone)

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32
Q

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

A

Splenectomy

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33
Q

Are ‘warm Abs’ IgM or IgG?

A

IgG

*Only IgG abs respond to steroids and splenectomy

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34
Q

Buzzword

Mycoplasma or EBV associated anemia

A

Cold-induced hemolysis

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35
Q

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

A

Negative

Complement test will be positive

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36
Q

How is cold-induced hemolysis treated?

A

Rituximab

*No response to steroids, splenectomy, or IVIG

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37
Q

Is cold-induced hemolysis IgG or IgM?

A

IgM

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38
Q

What is mode of inheritance of G6PD deficiency?

A

X-linked recessive

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39
Q

What are common triggers of G6PD deficiency anemia?

A

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

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40
Q

Buzzwords

Heinz bodies and bite cells are seen in this anemia

A

G6PD deficiency

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41
Q

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

A

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

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42
Q

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

A

Hereditary spherocytosis

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43
Q

What is the most accurate diagnostic test for hereditary spherocytosis?

A

Osmotic fragility test

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44
Q

Tx of hereditary spherocytosis

A

Splenectomy

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45
Q

Buzzword

E. Coli 0157:H7

A

Hemolytic-Uremic Syndrome

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46
Q

Ticlodipine can predispose to what macroangiopathic hemolytic anemia?

A

Thrombotic thrombocytopenic purpura (TTP)

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47
Q

Findings in HUS

A

Intravascular hemolysis
Elevated BUN and creatinine
Thrombocytopenia

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48
Q

Findings in TTP (5)

A 
Intravasacular hemolysis
Elevated BUN and creatinine
Thrombocytopenia
Fever
Neurological abnormalities
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49
Q

ADAMTS-13 level in TTP is …

A

Low

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50
Q

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

A

No

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51
Q

Severe cases of TTP or HUS are treated with what?

A

Plasmapharesis

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52
Q

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

A

Paroxysmal nocturnal hemoglobinuria

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53
Q

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

A

Large vessel thrombosis

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54
Q

Most accurate test for diagnosis of PNH

A

CD55 or CD59 Ab (aka decay accelerating factor)

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55
Q

Best initial tx of PNH

A

Steroids

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

56
Q

What are some drugs which may cause methemoglobinemia?

A

Nitroglycerin, amyl nitrate, nitroprusside dapsone, -caines

57
Q

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

A

Methemoglobinemia

Methylene blue

58
Q

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

A

IgA deficiency

59
Q

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

A

Minor blood grouping incompatability

60
Q

Buzzwords

Auer rods associated with

A

AML

61
Q

Buzzwords

DIC associated leukemia

A

M3, acute promyelocytic leukemia

62
Q

Best initial test in diagnosis of acute leukemia

A

PBS showing blasts

63
Q

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

A

Cytogenetic abnormalities suggesting high rate of relapse

64
Q

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

A

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

65
Q

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

A

Leukapheresis and hydroxyurea to reduce WBC count

66
Q

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

A

Myelodysplasia

67
Q

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

A

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

68
Q

Buzzword

Pelger-Huet cells association

A

Myelodysplasia

*The cell is a neutrophil with two lobes

69
Q

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

A

Infection or bleeding

70
Q

Tx of myelodysplasia

A

Supportive with transfusions as needed

*Azacitadine (increases survival), decitabine, and lenalidomide

71
Q

Why do patients have early satiety in CML and CLL?

A

Splenomegaly may compress stomach

72
Q

What happens to the following values in CML?

Leukocyte alkaline phosphatase (LAP)
Neutrophils

A

LAP decreases

Neutrophils increase

73
Q

Elevated LAP score indicates …

A

Leukomoid reaction (reactive increase in WBC)

74
Q

Buzzword

Philadelphia chromosome

A

bcr/abl in CML responding to Gleevac (imatinib)

75
Q

Best initial therapy CML

A

Imatinib

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

76
Q

Best initial test for CLL

A

PBS showing lymphocyte smudge cells

77
Q

Tx for CLL

A

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

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

78
Q

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

A

No

79
Q

If fludarabine fails in CLLthen what med is used?

A

Alemtuzumab (anti-CD52 agent)

80
Q

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

A

Hairy cell leukemia

81
Q

Best initial therapy in hairy cell leukemia

A

Cladribine (2-CDA)

82
Q

How can myelofibrosis and hairy cell leukemia be distinguished?

A

Hairy cell leukemia has elevated TRAP

Normal TRAP in myelofibrosis

83
Q

Buzzword

Tear drop cells

A

Myelofibrosis

84
Q

What mutation is seen in myelofibrosis?

A

JAK2

*Ruxolitinib inhibits JAK2 and is occasionally used

85
Q

Best initial therapy for myelofibrosis

A

Lenalidomide or thalidomide

86
Q

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

A

Polycythemia vera

87
Q

MCV in polycythemia vera

A

Low

88
Q

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

A

Polycythemia vera

89
Q

Best initial therapy for polycythemia vera

A

Phlebotomy

90
Q

Meds used for polycythemia vera and essential thrombocythemia

A

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

91
Q

What is erythromelalgia?

A

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

92
Q

What are most common causes of death of multiple myeloma?

A

Renal failure and infection

93
Q

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

A

Multiple myeloma

94
Q

Buzzwords

Bence-Jones protein on UPEP

A

Multiple myeloma

95
Q

How will a peeripheral smear appear in multiple myeloma?

A

Rouleaux formation from RBC clumping in chains

96
Q

What is a prognostic indicator in multiple myeloma?

A

Beta-2 microglobulin

97
Q

What medicine reverses the renal dysfunction in multiple myeloma?

A

Bortezomib

98
Q

B12 and LAP levels are changed how in polycythemia vera?

A

Both elevated

99
Q

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

A

Bone marrow biopsy

100
Q

Tx of multiple myeloma

A

Melphalan and steroids

Thalidomide, bortezomib, and lenalidomide may be added

101
Q

Patients that are relatively young (

A

Autologous stem cell BM transplantation

102
Q

Patients with hypercalcemia in multiple myeloma should be managed how?

A

Hydration and diuresis

103
Q

Asymptomatic elevation of IgG on SPEP

A

Monoclonal gammopathy of unknown significance

104
Q

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

A

Serum viscosity test

SPEP with elevated IgM

105
Q

Best initial therapy for symptomatic patients with Waldenstrom’s

A

Plasmapharesis

additional tx are rituximab fludarabine, chlorambucil, or rituximab

106
Q

Chronic Hep B and C can cause …

A

Aplastic anemia

107
Q

What are therapy options for patients with aplastic anemia?

A

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

108
Q

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

A

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

109
Q

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

A

Excisional lymph node biopsy

110
Q

Histologically how do Hodgkin’s and NHL differ

A

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

111
Q

Describe the staging scheme for lymphoma

A

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
Q

What other tests are helpful in staging lymphoma?

A

CXR
BM biopsy
CT scan with contrast

113
Q

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

A

Stages I and II: Radiotherapy and chemo

Stage III and IV: Chemo

114
Q

Tx for Hodgkin’s Disease

A

ADVB

Adriamycin, bleomycin, vinblastine, dacarbazine

115
Q

Tx for NHL

A 
CHOP
Cyclophosphamide
Hydroxyadriamycin
Oncovin (vincristine)
Prednisone

If anti-CD20 positive then add Rituximab

116
Q

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

A

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

117
Q

What are the most accurate tests for diagnosing vWD?

A

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

118
Q

What is first-line treatment for vWD?

A

Desmopression (DDAVP)

119
Q

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

A

Factor VIII replacement (contains both vWD and factor VIII)

120
Q

Antibodies to what receptor cause ITP?

A

Antibodies to glycoprotein IIb/IIIa receptor

121
Q

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

A

ITP

122
Q

If ITP is suspected what is the best next step?

A

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

123
Q

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

A

Provide IVIG

124
Q

What do romiplostim and eltrombopag do?

A

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

125
Q

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

A

Uremia-induced platelet dysfunction

126
Q

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

A

Their ability to degranulate

127
Q

Tx of uremia-induced platelet dysfunction

A

DDAVP, dialysis, estrogen

128
Q

What is treatment for factor XII deficiency?

A

No treamtent needed

129
Q

What is treatment for factor XI deficiency?

A

Fresh frozen plasma

130
Q

Does thrombosis in HIT occur more in veins or arteries?

A

Veins

131
Q

Best initial diagnostic tests for HIT

A

Platelet factor 4 Abs or heparin-induced platelet antibodies

132
Q

Buzzword

Russel viper venom test

A

Most accurate test for lupus anticoagulant

133
Q

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

A

Elevated aPTT
Normal PT
False positive VDRL

134
Q

Tx of APAS

A

Heparin followed by warfarin

135
Q

How does protein C deficiency present when starting anticoagulation?

A

Skin necrosis when patients go on warfarin

Venous thrombosis

136
Q

What is treatment of protein c deficiency?

A

Heparin followed by warfarin

137
Q

What is MOA of heparin?

A

Inactivates thrombin and activated Xa

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