B3-049 Disorders of Coagulation Flashcards
1
Q
VWF is synthesized from
A
endothelial cells (stored in Weibel Palade bodies)
platelets (stored in alpha granules)
2
Q
multimers that vary in molecular weight make up
A
VWF
3
Q
VWF is processed in the plasma by
A
ADAMTS-13
4
Q
protease that cleaves ultra large VWF multimers into smaller sizes
A
ADAMTS-13
5
Q
functions in platelet adhesion and stabilization of factor VIII
A
VWF
6
Q
fibrin meshwork stabilized by crosslinking
A
secondary hemostasis
7
Q
platelet plug formation
A
primary hemostasis
8
Q
factors of the extrinsic pathway
A
tissue factor
factor VII
9
Q
factors of the intrinsic pathway
A
prekallikrein
HMWK
XII
XI
IX
VIII
10
Q
factors of the common pathway
A
X
V
II (prothrombin)
I (fibrinogen)
11
Q
fibrin stabilization factor
A
XIII
12
Q
Vitamin K dependent coagulation proteins
A
II
VII
IX
X
C
S
13
Q
required for post-translation gamma carboxylation of glutamic acid residues
forming gla
A
vitamin K
14
Q
PFA-100 is screening test for which pathway
A
primary hemostasis
15
Q
PTT is a screening test for which pathway
A
intrinsic
16
Q
PT is a screening test for which pathway
A
extrinsic
17
Q
causes of abnormal PT
A
liver disease
vitamin K deficiency
warfarin
18
Q
how to calculate INR
A
19
Q
common causes of abnormal PTT
diseases
A
hemophilia A and B
vW disease
heparin
severe liver disease
severe vitamin K deficiency
warfarin
20
Q
causes of prolonged thrombin time
molecular level
A
Low fibrin
dysfibrinogenemia
thrombin inhibitors
fibrin monomer polymerization inhibitors
21
Q
fibrin degradation can be quantified using
A
D-dimer
22
Q
D dimer has [high/low] sensitvitiy
A
high for DIC
low for inhierited disorders of increased fibrinolysis
23
Q
D dimer has [high/low] specificity
A
low
positive in any disorder that produces intravascular clot
24
Q
D-dimer has a [high/low] negative predictive value
A
high for PE
25
bleeding within skin or mucous membranes
purpura
26
pinpoint bleeding of skin or mucous membranes
petechiae
27
large confluent area of bleeding within skin, larger than petechiae
ecchymosis
28
a collection of blood in an organ, space or tissue
hematoma
29
bleeding into the joint space
hemarthrosis
30
bleeding patterns consistent with primary hemostasis problem
skin/mucosal bleeds
purpura, petechiae, eccymosis
spontaneous bleeding
31
bleeding patterns consistent with an issue in secondary hemostasis
bleeds into soft tissue, muscle, joints
hemarthrosis
bleeding with trauma
32
partial quantititative deficiency
type 1 von willebrand
33
inheritance pattern of type 1 vWD
autosomal dominant
34
type 2vWD is [qualitative/quanitative]
qualitative
35
type 3 vWD is [quanitative/qualitative]
complete quantitative deficiency
36
inheritance pattern type 3 vWD
autosomal recessive
37
prolonged closure time on PFA-100
prolonged PTT
vWD
38
petechiae
ecchymosis
prolonged bleeding after tooth extraction
| associated with what disease
vWD
| hemarthrosis and hematomas in type 3 as well
38
petechiae
ecchymosis
prolonged bleeding after tooth extraction
| associated with what disease
vWD
| hemarthrosis and hematomas in type 3 as well
39
VWF concetrate, blood derivative (pathogen activated)
| vWD treatment
humate P
40
good source of vWF, blood product (not pathogen activated)
| vWD treatment
cryoprecipitate
41
stimulates endothelial cells to release remaining vWF stores
| vWD treatment
desmopressin
42
venous thrombotic disorders are
inherited or acquired
43
arterial thromotic disorders are caused by
platelet and vessel wall abnormalities
44
major acquired risk factors for venous thromboembolism
major surgery/trauma
hx of thrombosis
lupus anticoagulant
pregnancy
cancer
45
anatomic locations of DVT/VTE
deep veins of legs
pulmonary arteries
cerebral, mesenteric, hepatic veins (Budd-chiari)
46
diagnosis of DVT/VTE
compression ultrasound of proximal leg veins
D- dimer
Venography
| +1 if non compressible
47
pain, swelling, ulceration of affected leg
post thrombotic syndrome
| DVT/VTE
48
clinical features of PE
dyspnea, wheezing
pleuritic chest pain
hemoptysis
right sided heart failure
hypoxemia
49
diagnosis of PE
spiral CT- high PPV
D dimer- high NPV
ventilation perfusion scan
pulmonary angiography
50
point mutation leading to decreased proteolytic inactivation by protein C
Factor V Leiden
51
Factor V Leiden has a high prevalence in
caucasians
52
activated protein C resistance
Factor V Leiden
53
point mutation in 3' UTR leading to instability of mRNA
prothrombin gene mutation
54
causes increased prothrombin levels
| disease
prothrombin gene mutation
55
prothrombin gene mutation has a high prevalence in
caucasians
56
decreased activation of FVa and FVIIIa
protein C and protein S deficiency
57
warfarin induced skin necrosis is caused by
heterozygous protein C and S deficiency
antithrombin deficiency
58
neonatal purpura fulminans is caused by
homozygous protein C and protein S deficiency
59
decreased plasma protein C or protein S level
protein C and protein S deficiency
60
decreased in vitamin K deficiency
protein C and S
61
decreased inactivation of FX, FII
antithrombin deficiency
62
resistant to heparin therapy
antithrombin deficiency
63
homozygous state is incompatible with life
antithrombin deficiency
64
decreased plasma antithrobin level
antithrombin deficiency
65
autoantibodies against phospholipid binding proteins
antiphospholipid syndrome
66
venous and arterial thrombosis
antiphospholipid syndrome
67
venous thrombosis
arterial thrombosis
recurrent spontaneous pregnancy loss
antiphospholipid syndrome
68
lab diagnosis of antiphospholipid syndrome
+anticardiolipin
or + anti-B2 glycoprotein I antibody
or + lupus anticoag test
69
treatment for antiphospholipid syndrome
anticoagulant therapy
70
autoantibodies against heparin-platelet factor 4 complex
| causes platelet inactivation and thrombosis
heparin induced thrombocytopenia
71
rank the types of heparin according to risk of
heparin induced thrombocytopenia
UFH>LMWH>fondaparinux
72
4 T's of heparin induced thrombocytopenia
Timing: 5-7 days after starting heparin
Thrombocytopenia- 50% decrease from baseline or below reference range
Thrombosis: venous or arterial
exclude oTher causes
73
diagnosis of heparin induced thrombocytopenia
clinical
Heparin-PF4 antibodies
heparin induced activation assay
74
treatment heparin induced thrombocytopenia
d/c heparin
use direct thrombin inhibitors
75
consumptive coagulopathy
DIC
76
major causes of DIC
infection related sepsis
ABO incompatible reaction
acute pancreatitis
septic abortion, AFE
APL
brain injury
trauma
77
Clinicial features:
bleeding
thrombosis
DIC
78
hyaline microthrombi of microvessels
DIC
79
platelet and fibrin rich thrombi
DIC
80
thrombocytopenia
prolonged PT, PTT
decreased fibrinogen
elevated D dimer
DIC
81
microangiopathic hemolysis
| shistocytes
DIC
82
differential for DIC
other causes of MAHA
other causes of thrombocytopenia
other causes of coagulopathy
83
thrombocytopenia
normal PT, PTT
normal fibrinogen
ITP, TTP, HUS, aHUS
84
thrombocytopenia
increased PT, PTT
normal fibrinogen
cirrhosis
85
thrombocytopenia
normal PT
elevated PTT
normal fibrinogen
heparin induced thrombocytopenia
86
treatment DIC
treat underlying disorder
blood products
| if bleeding predominant, antifibrinolytic
if clotting predominant, hepar
87
prolonged PTT indicates
presence of inhibitor
| think anticoagulant or dysfunctional platlets
88
warfarin therapy would causes a prolonged
PT
| ultimately, INR as well
89
if factors VIII or X caused a prolonged PTT, the 1:1 mix would show
correction of clotting time
90
most appropriate test to monitor warfarin therapy
INR
91
tests for these values are standardized to allow easy comparison across all labs
INR
factor levels
92
tests for these values are highly dependent on testing systems, methodologies and reagents
| cannot easily be compared across labs
PT
PTT
TT
93
screens for primary hemostatic defects involving platelets or vWF
PFA-100
94
factor V leiden is associated with
increased risk of venous thrombosis
| decreased degradation of Va
95
thrombin time measures
the conversion of fibrinogen to fibrin and its crosslinking
96
deficiency of fibrin will cause
prolonged thrombin time
97
if the D dimer is negative, exclude .... from the differential
PE
98
positive d dimer can indicate
DIC
fibrinolytic disorder
99
platelet adhesion to the vascular wall at the site of injury is primarily mediated by
Gp1b and vWF
100
mediates platelet aggregation through fibrinogen binding across all platelets
GpIIb-IIIa
101
most common inherited coagulopathy
vWD
102
vWF is caused by ..... deficiency of vWF
quantitative
103
vWF is exposed with
endothelial injury
104
vFW is reponsible for
platelet aggregation
| links gp1b to exposed collagen
105
links gp1b to exposed collagen
vWF
106
vWD causes ..... bleeding time
prolonged
107
how does vWF affected factor VIII?
decreased vFW causes FVIII to have shorter half life
108
vWD causes prolonged [PT/PTT]
PTT
| decreased FVIII leads to decreased activity of intinsic pathway
109
negative ristocetin test, but normal plasma can be added to cause aggregation
vWD
110
desmopressin can be used to treat
vWD
111
desmopressin causes
endothelial cells to release stored vWF
112
aortic stenosis can causes
acquired vWD
113
APL can cause
DIC
114
DIC causes excessive
bleeding and clotting
115
sepsis can cause
DIC
116
obstetric complications can cause
DIC
117
consumptive coagulopathy
DIC
118
oozing from IVs or arterial lines
petechiae, ecchymoses, mucocutaneous bleeding
DIC
119
DIC causes prolonged [PT/PTT}
both
120
vitamin K dependent factors
2, 7, 9, 10
C and S
121
causes of vitamin K deficiency
malabsorption
antibiotics
cirrhosis
122
vitamin K deficiency causes prolonged [PT/PTT]
PT
123
vitamin K deficiency can be treated with
fresh frozen plasma
vitamin K supplementation
124
result of autoantibodies against cardiolipin
antiphospholipid syndrome
125
antiphospholipid syndrome can be secondary to
RA
HIV
SLE
126
lupus anticoagulants inhibit
clotting in lab, but promote clotting in situ
127
antiphospholipid causes increased [PT/PTT]
PTT
128
anti-beta2 gp
antiphospholipid syndrome
129
often presents with arterial or venous thrombosis in young patient
antiphospholipid syndrome
130
polycythemia vera can increase risk of thrombosis due to
hypereviscosity
131
most common inherited thrombosis syndrome
factor V Leiden
132
inheritance pattern of Factor V Leiden
autosomal dominant
133
protein C is unable to destroy activated factor V
Factor V Leiden
134
Factor V Leiden mutation elminates binding spot for
protein C
135
in factor V Leiden, thrombin activity is [increased/decreased]
increased
136
in factor V Leiden, increased generation of thrombin and loss of negative feedback loop cause
increased risk of venous thrombosis
137
cause increased risk of thrombosis in Factor V Leiden
OCP and preganancy
smoking
prolonged immobilization
138
catalyzes the conversion of fibrinogen to fibrin
thrombin
139
leads to overproduction of thrombin --> increased risk of venous thrombosis
prothrombin mutation
140
protein C or S deficiency causes increased
factor Va and VIIIa activity --> increased risk of venouns thrombosis
141
in protein C and S deficiency, avoid treatment with
warfarin
| increases thrombotic effect
142
in protein C and S deficiency, administration of warfarin can cause
warfarin induced skin necrosis
143
homozygous protein C of S deficiency can cause what in newborns?
neonatal purpura fulminans
144
ATIII deficeincy causes
decreased inhibition of thrombin
145
most inherited thrombosis disorders follow what inheritance pattern?
autosomal dominant
146
heparin has not effect in
ATIII deficiency
| heparin must bind antithrombin to function
