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Rizk Review > Hematology > Flashcards

Hematology Flashcards

1
Q

Etiology of hemophilia A?

A

Deficiency in factor VIII

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2
Q

Etiology of hemophilia B?

A

Deficiency in factor IX

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3
Q

Change to PTT in hemophilia A and B?

A

Prolonged

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4
Q

Change to PT in hemophilia A and B?

A

Normal

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5
Q

Inheritance pattern of hemophilia A and B?

A

X-linked recessive

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6
Q

Clinical presentation for hemophilia A and B?

A

Joint hemorrhage; No excessive bleeding after minor cuts

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7
Q

Treatment of hemophilia A?

A

Human factor VIII concentrate

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8
Q

Treatment of hemophilia B?

A

Human factor IX concentrate

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9
Q

Treatment for mild cases of hemophilia A?

A

Desmopressin

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10
Q

Mechanism of action of desmopressin in treatment of hemophilia A?

A

Desmopressin causes release of VWF and factor VIII from endothelial tissues

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11
Q

Inheritance pattern of hemophilia C?

A

AR

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12
Q

Etiology of hemophilia C?

A

Factor 11 deficiency

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13
Q

Best treatment for hemophilia C?

A

Fresh frozen plasma

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14
Q

Change to PTT in hemophilia C?

A

Prolonged

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15
Q

Change to PT in hemophilia C?

A

Normal

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16
Q

Clinical presentation factor 12 deficiency?

A

No problem with bleeding (can undergo surgery without worry of excessive bleeding)

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17
Q

Change to PTT in factor 12 deficiency?

A

Extremely prolonged

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18
Q

Change to PT in factor 12 deficiency?

A

Normal

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19
Q

Inheritance pattern of factor 13 deficiency?

A

AR

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20
Q

Lab values seen in factor 13 deficiency?

A

Normal PT and PTT

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21
Q

Diagnostic test for factor 13 deficiency?

A

Urease clot solubility test

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22
Q

Best treatment for factor 13 deficiency?

A

Small amounts of fresh frozen plasma every 3–4 weeks

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23
Q

Condition associated with factor X deficiency?

A

Amyloidosis

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24
Q

Most common hypercoagulable hereditary state?

A

Factor V Leiden

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25
Q

Etiology of Factor V Leiden?

A

Activated Protein C resistance

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26
Q

Best treatment for Factor V Leiden?

A

Indefinite warfarin

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27
Q

Medication that is contraindicated in Factor V Leiden?

A

OCPs

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28
Q

3 natural anti-coagulants that may cause thrombotic disorders if deficient?

A

Protein C, Protein S, Anti-thrombin III

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29
Q

33 yo female with known history of SLE; no history of miscarriage or abnormal arterial or venous clotting - what is next best step for treatment?

A

Observe

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30
Q

What type of thrombosis is associated with homocystinuria?

A

Both venous and arterial

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31
Q

Primary hemostasis is mediated by …

A

Platelets

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32
Q

Secondary hemostasis is mediated by …

A

Coagulation factors

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33
Q

Etiology of ITP?

A

IgG directed against GPIIb/IIIa on platelet surface

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34
Q

Coomb’s test for ITP?

A

(+)

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35
Q

Lab change associated with ITP?

A

Thrombocytopenia

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36
Q

Clinical presentation of ITP?

A

Thrombocytopenia in adolescents following viral URI

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37
Q

Diagnostic test required for ITP in asymptomatic patient with isolate thrombocytopenia?

A

Review peripheral blood smear … no further workup required

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38
Q

Best treatment for ITP in asymptomatic patient?

A

Supportive care

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39
Q

Best treatment for ITP in symptomatic patient?

A

Corticosteroids

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40
Q

5 aspects of clinical presentation associated with TTP?

A

FTKCS … Fever, thrombocytopenia, Kidney failure, Confusion, schistocytes

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41
Q

Change to PT and PTT in TTP?

A

NML

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42
Q

Change to LDH in TTP?

A

Elevated … due to RBC hemolysis

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43
Q

Medication associated with TTP?

A

Ticlopidine … grandfather of clopidogrel

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44
Q

Additional AE of Ticlopidine?

A

Agranulocytosis

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45
Q

Best treatment for TTP?

A

Plasmapheresis (plasma exchange)

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46
Q

Best treatment for TTP in patients in which plasmapheresis is not available?

A

FFP

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47
Q

Coomb’s test for TTP?

A

(-)

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48
Q

Change to PT and PTT in HUS?

A

NML

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49
Q

3 aspects of clinical presentation for HELLP syndrome?

A

Hemolytic anemia, Elevated LFTs, Low Platelets

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50
Q

Appearance of blood smear in HELLP syndrome?

A

Schistocytes

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51
Q

Change to PT and PTT in HELLP?

A

NML

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52
Q

Major complication of HIT?

A

Thrombosis … (not bleeding)

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53
Q

Best treatment for HIT?

A

Stop heparin … begin Argatroban

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54
Q

MOA of Argatroban?

A

Direct thrombin inhibitor

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55
Q

Inheritance pattern of Von Willebrand Disease?

A

AD

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56
Q

Lab change seen in setting of Von Willebrand Disease?

A

Prolonged bleeding time, Prolonged PTT

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57
Q

2 functions of Von Willebrand Factor?

A

Adhesion of platelets; Carrier protein for Factor VIII

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58
Q

Definitive diagnosis for Von Willebrand Disease?

A

Von Willebrand Factor assay

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59
Q

Best treatment for mild cases of Von Willebrand Disease?

A

DDVAP (vasopressin)

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60
Q

MOA of DDVAP (vasopressin) in treatment of Von Willebrand Disease?

A

Enhances release of VWF and Factor VIII from endothelial cells

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61
Q

Etiology of Bernard-Soulier Syndrome?

A

Deficiency in GPIb

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62
Q

Lab change associated with Bernard-Soulier Syndrome?

A

Low platelet count

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63
Q

Etiology of Glanzman Thrombasthenia?

A

Deficiency in GPIIb/IIIa

64
Q

Glanzman Thrombasthenia mimics the effects of which drug?

A

Abciximab

65
Q

Change to platelet count in setting of Glanzman Thrombasthenia?

A

NML

66
Q

Inheritance pattern of Glanzman Thrombasthenia?

A

AR

67
Q

Inheritance pattern of Bernard-Soulier Syndrome?

A

AR

68
Q

Change to PT and PTT in DIC?

A

Prolonged

69
Q

Coomb’s test for HUS?

A

(-)

70
Q

Coomb’s test for HELLP?

A

(-)

71
Q

Coomb’s test for DIC?

A

(-)

72
Q

Coomb’s test for Microangiopathic Anemia?

A

(-) … RBC fragmentation is mechanical … (not immunologic)

73
Q

4 conditions that feature schistocytes?

A

HUS, TTP, HELLP, DIC

74
Q

Alternate name for acanthocytes?

A

Spur cells

75
Q

Condition associated with acanthocytes?

A

Liver disease, Abetalipoproteinemia

76
Q

Inheritance pattern of Abetalipoproteinemia?

A

AR

77
Q

Etiology of Abetalipoproteinemia?

A

Lipid malabsorption by enterocytes

78
Q

Clinical presentation of Abetalipoproteinemia?

A

Diarrhea

79
Q

Condition associated with burr cells?

A

Kidney disease

80
Q

Which drug may cause sideroblastic anemia?

A

Isoniazid

81
Q

Connection between ETOH abuse + Isoniazid and sideroblastic anemia?

A

Both decrease Vitamin B6

82
Q

Patient presents with sideroblastic anemia on blood smear; Denies HX of ETOH use and Isoniazid therapy – next step of workup?

A

Check lead levels, erythrocyte protoporphyrin

83
Q

___ refers to deposition of lead sulfide along teeth and gingiva, seen in setting of lead poisoning

A

Burton’s line

84
Q

Where does lead typically deposit in bones?

A

Metaphysis

85
Q

Condition associated with teardrop-shaped RBCs?

A

Myelofibrosis

86
Q

Alternate name for RBCs that are teardrop-shaped?

A

Pyriform

87
Q

Pathogen that is teardrop-shaped?

A

Trichophyton rubrum

88
Q

Classic cell type seen in Myelodysplasia?

A

Pseudo-Pelger-Huet

89
Q

Description of Pseudo-Pelger-Huet cells?

A

Bilobed PMNs

90
Q

Conditions associated with Target cells?

A

Hemoglobinopathies, asplenia

91
Q

Inheritance pattern of G6PD deficiency?

A

X-linked recessive

92
Q

Etiology of bite cells in G6PD deficiency?

A

Attempt of spleen to remove Heinz bodies

93
Q

Change to serum iron in iron deficiency anemia?

A

Low

94
Q

Change to TIBC in iron deficiency anemia?

A

High

95
Q

Change to transferrin saturation in iron deficiency anemia?

A

Low

96
Q

Change to ferritin in iron deficiency anemia?

A

Low

97
Q

Change to transferrin receptors in iron deficiency anemia?

A

High

98
Q

Change to serum iron in anemia of chronic disease?

A

Low

99
Q

Change to TIBC in anemia of chronic disease?

A

Low

100
Q

Change to transferrin saturation in anemia of chronic disease?

A

Low

101
Q

Change to ferritin in anemia of chronic disease?

A

High

102
Q

Change to transferrin receptors in anemia of chronic disease?

A

NML

103
Q

Most specific method of diagnosing iron deficiency anemia?

A

Bone marrow iron stain

104
Q

Next step of workup for iron deficiency anemia, if colonoscopy is (-)?

A

EGD

105
Q

Most common pathogen responsible for iron deficiency anemia?

A

Ancylostoma duodenale … (hookworm)

106
Q

Alternate name for Osler-Weber-Rendu Syndrome?

A

Hereditary Hemorrhagic Telangiectasia

107
Q

Inheritance pattern of Hereditary Hemorrhagic Telangiectasia?

A

AD

108
Q

1st step of treatment for iron deficiency anemia?

A

Oral iron supply

109
Q

2nd step of treatment for iron deficiency anemia?

A

IV iron

110
Q

Inheritance pattern of hereditary sideroblastic anemia?

A

X-linked

111
Q

Deficiency associated with sideroblastic anemia?

A

Vitamin B6

112
Q

2 medications associated with sideroblastic anemia?

A

Isoniazid, Chloramphenicol

113
Q

Renal disorder associated with lead poisoning?

A

Chronic interstitial nephritis with papillary necrosis; Type 2 RTA

114
Q

How can you distinguish between b thalassemia minor and a thalassemia trait?

A

b thalassemia minor = increased HbA2, HbF; a thalassemia trait = NML HBG electrophoresis

115
Q

2 types of normocytic anemia?

A

Anemia of chronic disease, Aplastic anemia

116
Q

2 conditions associated with Pure RBC Aplasia?

A

Parvo B19 infection, Thymoma

117
Q

Only type of inherited aplastic anemia?

A

Fanconi Anemia

118
Q

Inheritance pattern of Fanconi Anemia?

A

AR

119
Q

Portion of nephron that is affected by Fanconi Syndrome?

A

Proximal tubule

120
Q

Are transfusions from family members preferred or avoided for patients with aplastic anemia?

A

Avoided

121
Q

Why should transfusions from family members be avoided in patients with aplastic anemia?

A

Sensitize the patient to HLA antigens

122
Q

2 pathogens associated with Plastic Anemia?

A

Hepatitis B, Hepatitis C

123
Q

What prevents complement-mediated destruction of RBCs in Paroxysmal Nocturnal Hemoglobinuria?

A

Loss of Glycosyl phosphatidyl inositol (GPI) anchor protein

124
Q

Most common cause of death in Paroxysmal Nocturnal Hemoglobinuria (PNH)?

A

Venous thromboembolism (DVT, hepatic vein thrombosis)

125
Q

Hematologic malignancy associated with Paroxysmal Nocturnal Hemoglobinuria?

A

AML

126
Q

3 treatments of choice for Paroxysmal Nocturnal Hemoglobinuria?

A

Warfarin (protection against venous thromboembolism), Corticosteroids, Iron supplementation

127
Q

Which DOC in Paroxysmal Nocturnal Hemoglobinuria targets CD5 to minimize hemolysis?

A

Eculizumab

128
Q

AE of Eculizumab?

A

Increased risk of meningococcal infection

129
Q

3 possible signs of hemolysis on blood smear?

A

Schistocytes, Sickle cells, Spherocytes

130
Q

Change to LDH in setting of hemolytic anemia?

A

Increased

131
Q

Change to reticulocytes in setting of hemolytic anemia?

A

Increased

132
Q

Change to Haptoglobin in setting of hemolytic anemia?

A

Decreased

133
Q

Change to Indirect bilirubin in setting of hemolytic anemia?

A

Increased

134
Q

Most common cause of death in patients with Sickle Cell Anemia?

A

Acute chest syndrome

135
Q

Most common renal manifestation of Sickle Cell Anemia?

A

Isosthenuria … inability to concentrate urine

136
Q

When is treatment for Sickle Cell Anemia with hydroxyurea contraindicated?

A

Pregnant patients … treat with oxycodone + acetaminophen

137
Q

Best treatment for patients with Sickle Cell Anemia who develop aplastic anemia?

A

Folic acid supplementation

138
Q

MSK complication of Sickle Cell Anemia?

A

Femoral head osteonecrosis

139
Q

Best treatment for Sickle Cell patients with priapism?

A

Blood exchange transfusion

140
Q

Best treatment for otherwise healthy patients with priapism?

A

Aspiration of blood from corpus cavernosum + Pseudoephrine

141
Q

Coomb’s Test in Hereditary Spherocytosis?

A

(-)

142
Q

Alternate name for spherocytes in Hereditary Spherocytosis?

A

Polychromatophilic RBCs

143
Q

Best treatment for symptomatic patients with Hereditary Spherocytosis?

A

Splenectomy

144
Q

Best test for patient with suspected Autoimmune Hemolytic Anemia (AIHA)?

A

Coomb’s Test … (direct antiglobulin test)

145
Q

Location of Ig in (+) Direct Coomb’s Test?

A

On RBC membrane

146
Q

Location of Ig in (+) Indirect Coomb’s Test?

A

In serum

147
Q

Ig associated with warm AIHA?

A

IgG

148
Q

Warm AIHA is ___vascular

A

Extra

149
Q

Warm AIHA is intra___

A

Splenic

150
Q

2 conditions associated with Warm AIHA?

A

SLE, Lymphoma

151
Q

Best treatment for Warm AIHA?

A

Corticosteroids + Splenectomy

152
Q

Ig associated with cold AIHA?

A

IgM

153
Q

Cold AIHA is ___vascular

A

Intra

154
Q

Cold AIHA is intra___

A

Hepatic

155
Q

4 conditions associated with Cold AIHA?

A

Malignancy, Mono, Mycoplasma, PNH

156
Q

Which type of AIHA is complement-mediated?

A

Cold

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