B3-066 CBCL Thrombocytopenia Flashcards
1
Q
small non-nucleated blood cells derived from bone marrow megakaryocytes
A
platelets
2
Q
alpha granules
A
platelet factor V
fibrinogen
VWF
PDGF
3
Q
dense granules
A
ADP
serotonin
calcium
4
Q
A
normal peripheral blood smear
5
Q
A
normal bone marrow
6
Q
Gplb deficiency
A
Bernard-Soulier
7
Q
GpIIb-IIIa deficiency
A
Glanzmann
8
Q
platelet function tests
A
PFA-100
platelet aggregation test
bleeding test
9
Q
test of platelet function that measures both platelet adhesion and aggregation
A
PFA test
10
Q
Normal CT Collagen-ADP
elevated CT Collagen EPI
A
aspirin/NSAIDs
11
Q
Normal or elevated CT Collagen-ADP
normal or elevated CT Collagen EPI
A
clopidogrel
12
Q
elevated CT Collagen-ADP
elevated CT Collagen EPI
3
A
VWD
Bernard Soulier
Glanzmann
13
Q
measures the ability of various agonists to platelets to induced in vitro activation and platelet-platelet activation
A
LTA
14
Q
LTA: when agnoist is added
A
platelets aggregate and absorb less light
15
Q
LTA: aggregation present only with ristocetin
A
glanzmann
16
Q
LTA: aggregation is absent with high concentrations of ristocetin
A
Bernard Soulier
17
Q
causes of thrombocytopenia
A
decreased platelet production
decreased platelet survival
sequestration in spleen
dilution
18
Q
A
thrombocytopenia
19
Q
A
aplastic anemia
20
Q
A
metastatic carcinoma
21
Q
A
ITP
foamy histiocytes in spleen
22
Q
A
TTP
shistocytes
23
Q
symptoms of thrombocytopenia
A
petechiae
ecchymoses
gum/nose bleeding
GI bleeding, hematuria, excessive menstrual flow
24
Q
inherited platelet disorders
A
bernard-soulier
glanzmann
disorders of platelet sectretion
25
defective adhesion
bernard-soulier
26
defective platelet aggregation
glanzmann
27
acquired defects of thrombocytopenia
aspirin
uremia
28
typically occuring in females
TTP
29
typically occuring in children
HUS
30
inhibition/deficiency of ADAMTS13 causes decreased degradation of vWF multimers
TTP
31
triad of thrombocytopenia, MAHA, and acute kidney injury
TTP and HUS
32
triad+ fever+ neurologic symptoms
TTP
33
triad+ blood diarrhea
HUS
34
normal PTT and PT
| disease processes
HUS/TTP
| distinguishes from DIC
35
treatment TTP
plamaphoresis
glucocorticoids
rituximab
36
treatment HUS
supportive
37
in patients with renal failure, uremic toxins accumulate and interfere with platelet adhesion
uremic platelet dysfunction
38
destruction of platelets in spleen
anti GpIIb/IIIa antibodies cause splenic macrophages to injest platelets
ITP
39
may by idiopathic or secondary to autoimmune disorders
ITP
| SLE, HIC, HCV, CLL
40
increased megakaryocytes on bone marrow biopsy, decreased platelet count
ITP
41
treatment ITP
glucocorticoids
IvIg
rituximab
42
blood smear shows no platelet clumping
glanzmann
43
prolonged PT and PTT
decreased platelets
decreased fibrinogen
elevated D dimer
DIC
44
thrombocytopenia and anti-platelet antibodies
ITP
45
what clinical feature is most likely to be seen in ITP?
petechiae
46
classic pentad of TTP
fever
thrombocytopenia
MAHA
neurologic abnormalities
renal failure
47
TTP is caused by autoantibodies that inhibit
ADAMTS13
48
vWF cleaving protease
ADAMTS13
49
anti-ADAMTS13 antibodies cause
TTP
50
anti beta 2 glycoprotein I antibodies cause
antiphospholipid antibody syndrome
51
anti factor VIII antibodies cause
acquired factor VIII deficiency
52
anti-platelet 4 antibodies cause
HIT
53
anti-red cell antibodies cause
AIHA
54
Glanzmanns does not cause
thrombocytopenia
| normal platelet count
55
4 causes of thrombocytopenia
splenomegaly
aplastic anemia
ITP
DIC
56
What virus can cause
marrow suppression
autoimmune antibodies to GpIIb-IIIa
deposition of immune complexes on platelets
thrombotic microangiopathy
HIV
57
diseases due to defective platelet function
| 4
glanzmanns
uremia
bernard soulier
storage pool diseases
58
immune mediated platelet destruction
ITP
59
metastatic carcinoma
aplastic anemia
acute myeloid leukemia
myelodysplastic syndromes
would all cause.....
thrombocytopenia
60
grey platelets
storage pool diseases
61
large platelets on peripheral blood smear
shistocytes on peripheral blood smear
microthrombi in glomerular arterioles
increased megakaryocytes in marrow
HUS
62
HUS is commonly caused by ...... in children
Shiga toxin
63
most effective treatment for HUS
supportive care
64
most common pathogen causing HUS
E. coli O157:H7
65
HIT is caused by
IgG antibodies that bind to multimolecular complexes of platelet factor 4 bound to heparin
66
absence of shistocytes on blood smear excludes
aHUS and TTP
67
normal coagulation tests exclude
vWD
68
negative medication history excludes
drug induced thrombocytopenia
69
ITP is a diagnosis by
exclusion
70
autoantibodies against GIIb-IIIa or Ib-IX cause
ITP
71
treatment for chronic ITP in adults
splenectomy
glucocorticoids
IvIg
rituximab
72
small lymphocytes with mature and condensed chromatin, smudge cell= CLL
ITP is commonly associated with CLL
73
commonly associated with ITP
| cancer
CLL
74
mucosal bleeding is most characteristic of a
platelet disorder
75
absent platelet aggregation in response to bovine or human vWF + ristocetin
bernard soulier
76
abnormal GPIb-IX
bernard soulier
77
giant platelets with thrombocytopenia
bernard soulier
78
abnormal platelet aggregation in response to ADP, collagen, and epinephrine
glanzmanns
79
abnormal gpIIb-IIIa
glanzmanns
80
thrombocytopenia
fever
renal disease
MAHA
neurologic complications
hallmarks of
TTP
81
in qualitative platlet dysfunction bleeding time is
prolonged
82
platelet dysfunction causes what type of bleeding?
bruises, petechiae, epistaxis
83
uremia causes [quantitative or qualitative] platelet dysfunction
qualitative
84
trauma causes [quantitative/qualitative] platelet dysfunction
qualitative
85
bernard soulier is a [qualitative/quantitative] platelet dysfunction
qualitative
86
inheritance pattern of bernard soulier
autosomal recessive
87
caused by deficiency of gp1b receptors
bernard soulier
88
responsible for adhering platelets to vWF
gp1b
89
absence of bp1b in bernard soulier causes
no platelet formation, bleeding
90
giant platelets in blood
bernard soulier
91
ristocein test is negative
bernard soulier
92
glanzmann is a [quantitative or qualitative] platelet dysfunction
qualitative
93
inheritance pattern glanzmann
autosomal recessive
94
in ristocetin test for bernard-soulier, addition of normal serum does
NOT lead to platelet aggregation
95
dysfunctional gp2a3b
glanzmann
96
the dysfunctional gp3a2b in glanzmann causes
fibrinogen to be unable to bind --> reduced formation of platelet plug --> bleeding
97
thrombocytopenia is defined as a platelet count below
150,000
98
bleeding risk with thrombocytopenia doesnt begin until platelets fall below
50,000
99
spontaneous bleeding may occur when platelets fall below
20,000
100
how do quanitative disorders affect PT/PTT?
increase
101
how do qualitative platelet disorders affect PT/PTT?
normal
102
caused by IgG autoantibodies against gp2b3a
ITP
103
IgG coated platelets in ITP are
phagocytsed by macrophages in the spleen
104
ITP in children is usually
self limited
105
ITP in adults causes
chronic disease with acute flares
106
typically ITP affects
young women in 20s-30s
107
treatment of ITP
immunosuppresion, corticosteroids, IVIg
108
splenectomy is used to treat
refractory ITP
109
caused by development of autoanitbodies that bind platelet factor 4 heparin complex
HIT
110
antibody binding of platelets in HIT causes
degranulation
| release of cytokines, PF4 and ADP -->clotting
111
platelet activation in HIT leads to
platelet aggregation and clot formation
| DVT, PE
112
phagocytosis of IgG covered platelets in spleen
HIT, ITP
113
fever, chills, tachycardia, dyspnea
HIT
114
treatment of HIT
direct thrombin inhibitor
| bivalrudin, argatroban
115
cause diffuse platelet activation
TTP, HUS
116
Shiga-like toxin damages
capillary endothelial cells
117
triad of HUS
thrombocytopenia
MAHA
acute kidney injury
118
TTP is commonly seen in
young women
119
autoantibodies against ADAMTS13
TTP
120
ADAMSTS13 cleaves
large vWF complexes
121
neurologic symptoms caused by microvascular injury
TTP
122
splenomegaly/portal hypertension can increase
sequestration of platelets in spleen, causing thrombocytopenia
