Hematology Flashcards
(156 cards)
1
Q
Etiology of hemophilia A?
A
Deficiency in factor VIII
2
Q
Etiology of hemophilia B?
A
Deficiency in factor IX
3
Q
Change to PTT in hemophilia A and B?
A
Prolonged
4
Q
Change to PT in hemophilia A and B?
A
Normal
5
Q
Inheritance pattern of hemophilia A and B?
A
X-linked recessive
6
Q
Clinical presentation for hemophilia A and B?
A
Joint hemorrhage; No excessive bleeding after minor cuts
7
Q
Treatment of hemophilia A?
A
Human factor VIII concentrate
8
Q
Treatment of hemophilia B?
A
Human factor IX concentrate
9
Q
Treatment for mild cases of hemophilia A?
A
Desmopressin
10
Q
Mechanism of action of desmopressin in treatment of hemophilia A?
A
Desmopressin causes release of VWF and factor VIII from endothelial tissues
11
Q
Inheritance pattern of hemophilia C?
A
AR
12
Q
Etiology of hemophilia C?
A
Factor 11 deficiency
13
Q
Best treatment for hemophilia C?
A
Fresh frozen plasma
14
Q
Change to PTT in hemophilia C?
A
Prolonged
15
Q
Change to PT in hemophilia C?
A
Normal
16
Q
Clinical presentation factor 12 deficiency?
A
No problem with bleeding (can undergo surgery without worry of excessive bleeding)
17
Q
Change to PTT in factor 12 deficiency?
A
Extremely prolonged
18
Q
Change to PT in factor 12 deficiency?
A
Normal
19
Q
Inheritance pattern of factor 13 deficiency?
A
AR
20
Q
Lab values seen in factor 13 deficiency?
A
Normal PT and PTT
21
Q
Diagnostic test for factor 13 deficiency?
A
Urease clot solubility test
22
Q
Best treatment for factor 13 deficiency?
A
Small amounts of fresh frozen plasma every 3–4 weeks
23
Q
Condition associated with factor X deficiency?
A
Amyloidosis
24
Q
Most common hypercoagulable hereditary state?
A
Factor V Leiden
25
Etiology of Factor V Leiden?
Activated Protein C resistance
26
Best treatment for Factor V Leiden?
Indefinite warfarin
27
Medication that is contraindicated in Factor V Leiden?
OCPs
28
3 natural anti-coagulants that may cause thrombotic disorders if deficient?
Protein C, Protein S, Anti-thrombin III
29
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?
Observe
30
What type of thrombosis is associated with homocystinuria?
Both venous and arterial
31
Primary hemostasis is mediated by …
Platelets
32
Secondary hemostasis is mediated by …
Coagulation factors
33
Etiology of ITP?
IgG directed against GPIIb/IIIa on platelet surface
34
Coomb’s test for ITP?
(+)
35
Lab change associated with ITP?
Thrombocytopenia
36
Clinical presentation of ITP?
Thrombocytopenia in adolescents following viral URI
37
Diagnostic test required for ITP in asymptomatic patient with isolate thrombocytopenia?
Review peripheral blood smear … no further workup required
38
Best treatment for ITP in asymptomatic patient?
Supportive care
39
Best treatment for ITP in symptomatic patient?
Corticosteroids
40
5 aspects of clinical presentation associated with TTP?
FTKCS … Fever, thrombocytopenia, Kidney failure, Confusion, schistocytes
41
Change to PT and PTT in TTP?
NML
42
Change to LDH in TTP?
Elevated … due to RBC hemolysis
43
Medication associated with TTP?
Ticlopidine … grandfather of clopidogrel
44
Additional AE of Ticlopidine?
Agranulocytosis
45
Best treatment for TTP?
Plasmapheresis (plasma exchange)
46
Best treatment for TTP in patients in which plasmapheresis is not available?
FFP
47
Coomb’s test for TTP?
(-)
48
Change to PT and PTT in HUS?
NML
49
3 aspects of clinical presentation for HELLP syndrome?
Hemolytic anemia, Elevated LFTs, Low Platelets
50
Appearance of blood smear in HELLP syndrome?
Schistocytes
51
Change to PT and PTT in HELLP?
NML
52
Major complication of HIT?
Thrombosis … (not bleeding)
53
Best treatment for HIT?
Stop heparin … begin Argatroban
54
MOA of Argatroban?
Direct thrombin inhibitor
55
Inheritance pattern of Von Willebrand Disease?
AD
56
Lab change seen in setting of Von Willebrand Disease?
Prolonged bleeding time, Prolonged PTT
57
2 functions of Von Willebrand Factor?
Adhesion of platelets; Carrier protein for Factor VIII
58
Definitive diagnosis for Von Willebrand Disease?
Von Willebrand Factor assay
59
Best treatment for mild cases of Von Willebrand Disease?
DDVAP (vasopressin)
60
MOA of DDVAP (vasopressin) in treatment of Von Willebrand Disease?
Enhances release of VWF and Factor VIII from endothelial cells
61
Etiology of Bernard-Soulier Syndrome?
Deficiency in GPIb
62
Lab change associated with Bernard-Soulier Syndrome?
Low platelet count
63
Etiology of Glanzman Thrombasthenia?
Deficiency in GPIIb/IIIa
64
Glanzman Thrombasthenia mimics the effects of which drug?
Abciximab
65
Change to platelet count in setting of Glanzman Thrombasthenia?
NML
66
Inheritance pattern of Glanzman Thrombasthenia?
AR
67
Inheritance pattern of Bernard-Soulier Syndrome?
AR
68
Change to PT and PTT in DIC?
Prolonged
69
Coomb’s test for HUS?
(-)
70
Coomb’s test for HELLP?
(-)
71
Coomb’s test for DIC?
(-)
72
Coomb’s test for Microangiopathic Anemia?
(-) … RBC fragmentation is mechanical … (not immunologic)
73
4 conditions that feature schistocytes?
HUS, TTP, HELLP, DIC
74
Alternate name for acanthocytes?
Spur cells
75
Condition associated with acanthocytes?
Liver disease, Abetalipoproteinemia
76
Inheritance pattern of Abetalipoproteinemia?
AR
77
Etiology of Abetalipoproteinemia?
Lipid malabsorption by enterocytes
78
Clinical presentation of Abetalipoproteinemia?
Diarrhea
79
Condition associated with burr cells?
Kidney disease
80
Which drug may cause sideroblastic anemia?
Isoniazid
81
Connection between ETOH abuse + Isoniazid and sideroblastic anemia?
Both decrease Vitamin B6
82
Patient presents with sideroblastic anemia on blood smear; Denies HX of ETOH use and Isoniazid therapy – next step of workup?
Check lead levels, erythrocyte protoporphyrin
83
\_\_\_ refers to deposition of lead sulfide along teeth and gingiva, seen in setting of lead poisoning
Burton’s line
84
Where does lead typically deposit in bones?
Metaphysis
85
Condition associated with teardrop-shaped RBCs?
Myelofibrosis
86
Alternate name for RBCs that are teardrop-shaped?
Pyriform
87
Pathogen that is teardrop-shaped?
Trichophyton rubrum
88
Classic cell type seen in Myelodysplasia?
Pseudo-Pelger-Huet
89
Description of Pseudo-Pelger-Huet cells?
Bilobed PMNs
90
Conditions associated with Target cells?
Hemoglobinopathies, asplenia
91
Inheritance pattern of G6PD deficiency?
X-linked recessive
92
Etiology of bite cells in G6PD deficiency?
Attempt of spleen to remove Heinz bodies
93
Change to serum iron in iron deficiency anemia?
Low
94
Change to TIBC in iron deficiency anemia?
High
95
Change to transferrin saturation in iron deficiency anemia?
Low
96
Change to ferritin in iron deficiency anemia?
Low
97
Change to transferrin receptors in iron deficiency anemia?
High
98
Change to serum iron in anemia of chronic disease?
Low
99
Change to TIBC in anemia of chronic disease?
Low
100
Change to transferrin saturation in anemia of chronic disease?
Low
101
Change to ferritin in anemia of chronic disease?
High
102
Change to transferrin receptors in anemia of chronic disease?
NML
103
Most specific method of diagnosing iron deficiency anemia?
Bone marrow iron stain
104
Next step of workup for iron deficiency anemia, if colonoscopy is (-)?
EGD
105
Most common pathogen responsible for iron deficiency anemia?
Ancylostoma duodenale … (hookworm)
106
Alternate name for Osler-Weber-Rendu Syndrome?
Hereditary Hemorrhagic Telangiectasia
107
Inheritance pattern of Hereditary Hemorrhagic Telangiectasia?
AD
108
1st step of treatment for iron deficiency anemia?
Oral iron supply
109
2nd step of treatment for iron deficiency anemia?
IV iron
110
Inheritance pattern of hereditary sideroblastic anemia?
X-linked
111
Deficiency associated with sideroblastic anemia?
Vitamin B6
112
2 medications associated with sideroblastic anemia?
Isoniazid, Chloramphenicol
113
Renal disorder associated with lead poisoning?
Chronic interstitial nephritis with papillary necrosis; Type 2 RTA
114
How can you distinguish between b thalassemia minor and a thalassemia trait?
b thalassemia minor = increased HbA2, HbF; a thalassemia trait = NML HBG electrophoresis
115
2 types of normocytic anemia?
Anemia of chronic disease, Aplastic anemia
116
2 conditions associated with Pure RBC Aplasia?
Parvo B19 infection, Thymoma
117
Only type of inherited aplastic anemia?
Fanconi Anemia
118
Inheritance pattern of Fanconi Anemia?
AR
119
Portion of nephron that is affected by Fanconi Syndrome?
Proximal tubule
120
Are transfusions from family members preferred or avoided for patients with aplastic anemia?
Avoided
121
Why should transfusions from family members be avoided in patients with aplastic anemia?
Sensitize the patient to HLA antigens
122
2 pathogens associated with Plastic Anemia?
Hepatitis B, Hepatitis C
123
What prevents complement-mediated destruction of RBCs in Paroxysmal Nocturnal Hemoglobinuria?
Loss of Glycosyl phosphatidyl inositol (GPI) anchor protein
124
Most common cause of death in Paroxysmal Nocturnal Hemoglobinuria (PNH)?
Venous thromboembolism (DVT, hepatic vein thrombosis)
125
Hematologic malignancy associated with Paroxysmal Nocturnal Hemoglobinuria?
AML
126
3 treatments of choice for Paroxysmal Nocturnal Hemoglobinuria?
Warfarin (protection against venous thromboembolism), Corticosteroids, Iron supplementation
127
Which DOC in Paroxysmal Nocturnal Hemoglobinuria targets CD5 to minimize hemolysis?
Eculizumab
128
AE of Eculizumab?
Increased risk of meningococcal infection
129
3 possible signs of hemolysis on blood smear?
Schistocytes, Sickle cells, Spherocytes
130
Change to LDH in setting of hemolytic anemia?
Increased
131
Change to reticulocytes in setting of hemolytic anemia?
Increased
132
Change to Haptoglobin in setting of hemolytic anemia?
Decreased
133
Change to Indirect bilirubin in setting of hemolytic anemia?
Increased
134
Most common cause of death in patients with Sickle Cell Anemia?
Acute chest syndrome
135
Most common renal manifestation of Sickle Cell Anemia?
Isosthenuria … inability to concentrate urine
136
When is treatment for Sickle Cell Anemia with hydroxyurea contraindicated?
Pregnant patients … treat with oxycodone + acetaminophen
137
Best treatment for patients with Sickle Cell Anemia who develop aplastic anemia?
Folic acid supplementation
138
MSK complication of Sickle Cell Anemia?
Femoral head osteonecrosis
139
Best treatment for Sickle Cell patients with priapism?
Blood exchange transfusion
140
Best treatment for otherwise healthy patients with priapism?
Aspiration of blood from corpus cavernosum + Pseudoephrine
141
Coomb’s Test in Hereditary Spherocytosis?
(-)
142
Alternate name for spherocytes in Hereditary Spherocytosis?
Polychromatophilic RBCs
143
Best treatment for symptomatic patients with Hereditary Spherocytosis?
Splenectomy
144
Best test for patient with suspected Autoimmune Hemolytic Anemia (AIHA)?
Coomb’s Test … (direct antiglobulin test)
145
Location of Ig in (+) Direct Coomb’s Test?
On RBC membrane
146
Location of Ig in (+) Indirect Coomb’s Test?
In serum
147
Ig associated with warm AIHA?
IgG
148
Warm AIHA is \_\_\_vascular
Extra
149
Warm AIHA is intra\_\_\_
Splenic
150
2 conditions associated with Warm AIHA?
SLE, Lymphoma
151
Best treatment for Warm AIHA?
Corticosteroids + Splenectomy
152
Ig associated with cold AIHA?
IgM
153
Cold AIHA is \_\_\_vascular
Intra
154
Cold AIHA is intra\_\_\_
Hepatic
155
4 conditions associated with Cold AIHA?
Malignancy, Mono, Mycoplasma, PNH
156
Which type of AIHA is complement-mediated?
Cold
