Exam 2 Material Flashcards
(178 cards)
1
Q
Coagulation proteins in circulation that are inactive are called:
A
zymogens
2
Q
What is the purpose of activated coagulation proteins:
A
interact to form fibrin clots, reinforcing the platelet plug
3
Q
How were the coag proteins named:
A
based on sequence of discovery
4
Q
All coag factors are produced in the ___:
A
liver
5
Q
Factor 1:
A
Fibrinogen
6
Q
Fibrinogen is Factor___:
A
1
7
Q
Factor 2:
A
Prothrombin
8
Q
Prothrombin is Factor__:
A
2
9
Q
Tissue Factor is factor ___:
A
3
10
Q
Factor 3 is ____:
A
Tissue Factor
11
Q
Ionized calcium is factor___:
A
4
12
Q
Factor 4 is:
A
Ionized calcium
13
Q
Labile Factor is factor___:
A
5
14
Q
Factor 5 is ____:
A
Labile Factor
15
Q
Stable factor/proconvertin is factor___:
A
7
16
Q
Factor 7 is ___:
A
Stable factor/proconvertin
17
Q
Antihemophilic factor is factor___:
A
8
18
Q
Factor 8 is ___:
A
Antihemophilic factor
19
Q
Christmas factor is factor___:
A
9
20
Q
Factor 9 is _____:
A
Christmas factor
21
Q
Stuart-Prower factor is factor ___:
A
10
22
Q
Factor 10 is ____:
A
Stuart Prower Factor
23
Q
PTA is factor___:
Plasma Thromboplastin Antecedent
A
11
24
Q
Factor 11 is ___:
A
PTA
Plasma Thromboplastin Antecedent
25
Hageman Factor is factor ___:
12
26
Factor 12 is ___:
Hageman Factor
27
FSF is factor___:
| Fibrin Stabilizing Factor
13
28
Factor 13 is ____:
FSF
| Fibrin Stabilizing Factor
29
Fitzgerald Factor is ______:
HMWK
30
HMWK is _____ factor:
Fitzgerald Factor
31
Fletcher factor is ____:
Prekallikrein
32
Prekallikrein is ____ factor:
Fletcher
33
What is the main substrate in the coag cascade:
fibrinogen
34
List the two cofactors of the coag cascade:
* Factor 5
| * Factor 8:C
35
What do serine proteases do:
cut peptide bonds
36
Serine proteases include all but which factor:
Factor 8
37
_____ create cross linking in fibrin clot:
transaminases
38
_______ cut peptide bonds:
Serine proteases
39
Factor 8a is the only _____:
Transaminase
40
Contact proteins are only involved in initial phase of intrinsic activation and not consumed in normal clotting process, list the 4 of them:
11
12
Fitzgerald
Fletcher
41
Coumadin works ____, while heparin works _____:
Coumadin: in vivo
Heparin: in vivo and in vitro
42
List the Vit K dependent factors:
2,7, 9, 10
| Protein C & S
43
PT is used to monitor ____ therapy:
Coumadin/Warfarin
44
Acquired Vit K deficiences most commonly seen in the following:
* post-surgical
* High dose antibiotic use
* liver disease
* malnutrition
45
List the fibrinogen or thrombin sensitive proteins (i.e. thrombin acts upon them):
1, 5, 8:C, 13
46
____ acts on factors 1, 5, 8:C, and 13:
Thrombin
47
_____ acts on all factors of in the fibrinogen group:
Thrombin
48
Which factors have positive feedback, procoagulant effects with Thrombin:
Factors 5 and 8
49
How is Factor 1 converted to soluble fibrin monomer:
Fibrinopeptides A & B are cleaved
50
Coag inhibitor binds to antithrombin (AT), and presence of heparin enhances this binding by how much:
200 times
51
______ has procoagulant and coagulation inhibiting effects:
Thrombin
52
The procoagulant effects of Thrombin rely on ___ feedback with Factors 5 and 8:
positive
53
The coagulation inhibiting effects of Thrombin binds to Thrombomodulin, via ___feedback with Factors 5a and 8a:
negative feedback
| keeps clotting from getting out of control
54
Thrombin initiates tissue repair via these two mechanisms:
* induces chemotaxis
| * stimulates proliferation of smooth muscle and endothelial cells
55
Which anticoagulants affect the extrinsic pathway:
coumadin/warfarin
| test via PT
56
Why must calcium be added to a specimen to activate the coag cascade:
The citrate in a light blue top tube chelates the specimen calcium, so more must be added
57
Excess of PAI-1 is associated with _____:
thrombotic disease
| higher in MI patients >45
58
The primary substrate of PAI-1 is ___, thus regulation of fibrinolysis is dependent on the interaction of ___ with ____:
t-PA
| t-PA with PAI-1
59
T/F
| PAI-1 is an acute phase reactant that is synthesized by blood vessel endothelium and released in an inactive state:
True.
60
BT would be ____ in fibrinogen disorders because ____:
normal
| BT tests platelet function
61
_____ is a quantitative disorder causes by lack of synthesis in the liver:
afibrinogenemia
62
Levels of fibrinogen <100, generally asymptomatic:
hypofibrinogenemia
63
Abnormal structure and function of fibrinogen; qualitative disorder:
Dysfibrinogenemia
64
In dysfibrinogenemia, would BT and fibrinogen levels likely be normal:
Yes.
65
____ functions as a catalyst, forming bonds between proteins (fibrin monomers, fibronectin, collagen, alpha 2 inhibitor):
F13
66
___ deficiency is characterized by initial stoppage of bleeding, then recurrence of bleeding ~36 hours after event, low levels of this factor can be detected via ____:
Factor 13
| 5M urea test
67
List the 3 acquired disorders of secondary hemostasis:
* DIC
* Primary Fibrinolysis
* Liver disease
68
Why will the PT/APTT/ and TT be prolonged in DIC:
Due to consumption coagulopathy
69
What is the first and foremost treatment of DIC:
Remove stimulus!
then:
*LMWH, FFP, cryo
70
____ results from increased levels of plasmin:
Primary Fibrinolysis
71
What 3 things are normal in Fibrinolysis, but abnormal in DIC:
* d-dimer (high in DIC)
* Plt count (low in DIC)
* RBC's (fragments in DIC)
72
Factor ___ has the shortest half-life:
7
73
You'd see a decrease in ___ factors in mild liver disease:
vit k dependent factors
74
Plt dysfunction, including decreased plt adhesion, abnormal plt aggregation to ADP, epi, and thrombin, and abnormal PF3 availability, occur in this acquired disorder of secondary hemostasis:
liver disease
75
Patients with ___ will present with diffuse hemorrhages due to increased plasmin fibrinolytic activity, where they actively form clots that will dissolve in a couple hours:
Primary Fibrinolysis
76
Pregancy, cancer metastases, promyelocytic leukemia would be____ causes of DIC:
Extrinsic
77
Events that damage vascular endothelium and expose collagen such as infectious diseases, snake venom, massive trauma/surgery would be ____ causes of DIC:
Intrinsic
78
_____ is seen in 1/ of chronic liver disease patients due to spleen sequestration secondary to congestive splenomegaly:
Thrombocytopenia
79
Congjugated estrogen can be used as a treatment for thrombocytopenia caused by ____:
liver disease
80
Hemophilia A is a deficiency of ____, and is the most common hereditary coag disorder:
Factor 8:C
81
Hemophilia A exhibits this type of inheritance:
X-linked recessive
82
Severe hemophilia A has F8:C levels of ____ and requires constant transfusion therapy of ___:
<1%
| Factor 8 concentrates
83
Why is there an increased risk of developing alloantibodies or inhibitors to F8:C in Hemophilia A:
Due to regular F8:C infusions
84
Symptoms of alloantibodies to F8:C include:
hemarthrosis
hematuria
intracranial bleeds
hematomas
85
Why is a recombinant factor replacement preferred:
Risk of alloantibodies
86
In Hemophilia A, mixing studies do not correct with ____, which means either an inhibitor is present or reacting antibodies:
normal pool plasma
87
Which coag test will be abnorml in Hemophilila A:
APTT
| prolonged if F8<20%
88
Also known as Christmas Disease:
Hemophilia B (F9 deficiency)
89
Hemophilia B is a deficiency of factor__:
9
90
Factor 9 can be activated by ___:
11a+Ca, or Russell Viper Venom
91
T/F
| Hemophilia B is sex-linked and less common than Hemophilia A:
True
92
Which has a longer half-life, F8:C or F9 concentrates:
F9: 24 hrs
F8:C 12 hrs
93
Can Hemophilia B be acquired, and if so, how:
Yes.
| Liver disease, Vit K deficiency, oral antiocoagulant therapy
94
List the respective deficiency of each Hemophilia:
A:
B:
C:
A: 8:C
B: 9
C: 11
95
Which coag test would be abnormal in Hemophilia B, and would mixing studies correct with NPP and AP:
APTT (F9 is intrinsic)
| Yes
96
Aged serum contains which factors:
```
7
9
10
11
12
```
97
Adsorbed plasma has which factors removed:
Vit K dependent
98
Adsorbed plasma contains which factors:
```
1
5
8
11
12
```
99
Hemophilia C is also known as ____:
Rosenthal Syndrome
100
Rosenthal syndrome is also known as ______ and is predominantly in the ____ population:
Hemophilia C
| Ashkenazi Jewish population (1:8)
101
Bleeding after dental surgery/extraction in an Ashkenazi Jew, think ____:
Hemophilia C
102
Why is F11 replacement not needed in Hemophilia C unless patient is scheduled for surgery:
F11 is early in the cascade, and there are other ways it can be initiated
103
__or ____ possible in severe F11 deficiency:
alloantibodies or inhibitors
104
F11 levels >120% increase risk of ___:
thrombosis
105
Overabundance of F11 will bind to ___ binding sites, preventing ____ from activating, therefore preventing fibrinolysis, resulting in ____:
plasminogen
plasminogen
thrombosis
106
Which coag test will be abnormal in Hemophilia C:
APTT
107
vWD occurs almost as frequently as ____:
Hemophilia A
108
Will the BT be increased or decreased in vWD:
increased
109
Plt aggregation in vWD is impaired with ___:
Ristocetin
110
Factor 7 deficiency (aka Proconvertin) has symptoms similar to factor ___ deficiency:
8
111
Deficiency of factor___ will have a prolonged PT that will fully correct with RVV and mixing with aged serum:
Factor 7 deficiency
112
How does RVV correct a Factor 7 deficiency:
It activates Factor 10
113
T/F
Factor 10 deficiency is extremely rare, can occur at any age, and can be due to quantitative or qualitative abnormalities of the Factor 10 molecule:
True.
114
Is Stypven Time prolonged in Factor 10 deficiency:
Yes. (depends on F2, 5, 10 w/ phospholipid)
115
With the Factor 10 assay, variant forms of F10 could show ____ between tests:
discrepancies
116
___activity of F10 is considered adequate for hemostasis:
10%
117
Factor 5 deficiency is also known as ___:
parahemophilia
118
Factor 5 is also called _____ because of rapid deterioration in plasma at room temp:
labile
119
F5 is a catalyst in conversion of ___to ___:
F2-->F2a
120
Acquired F5 deficiency can occur from specific Ab acquired after ____ or use of ___ in surgery:
childbirth
| fibrin glue
121
1/2 of F5 deficient patients have an increased __ due to plt-related function of F5 of binding ___ to plt surface:
BT
| F10
122
In testing for a F5 deficiency, specimens must be ____:
platelet poor (<10k)
123
Deficiency of this delays generation of thrombin, causing severe hemorrhagic symptoms:
Factor 2 (Prothrombin)
124
The 'Prothrombin Complex' includes everything from ___ to ___:
F2 to Thrombin
125
Hypoprothrombinemia is a deficiency of this factor:
F2 (prothrombin)
126
Dysprothrombinemia is a structural defect of ___ that causes _____:
F2 (prothrombin)
| impaired activity
127
A single point mutation on chromosome 11 leads to a mutation of factor___ and is the 2nd most common cause of _____:
F2 (prothrombin)
| inherited thrombophilia
128
Does the Prothrombin Mutation on Chromosome 11 lead to an increased risk of clotting or bleeding:
clot risk
* risk factor for MI/stroke in young pts
* mostly caucasian
129
____deficiency is not associated with clinical bleeding or hemorrhage, patients are asymptomatic and post no surgical risk, even though their APTT is prolonged:
F12 (Hageman factor)
130
Deficiencies of Fletcher or Fitzgerald factors will both show marked prolongation of ____:
APTT
131
In Fletcher deficiency, contact activation time with APTT kaolin-like reagents changes with incubation time intervals, the APTT intervals will progessively ___:
shorten
132
Is there any racial predilection or apparent clinical bleeding associated with Fletcher or Fitzgerald factor deficiencies:
No.
133
One could acquire inhibition of factor____ following TB treatment with Isoniazid:
F13
134
Autoimmune inhibitors of F8:C most commonly result from___ and produce ___class of antibodies:
F8:C transfusions
IgG
(can also be seen in patients with RA, SLE, drug rx, post partum, etc)
135
Poor diet, biliary obstruction, malabsorption, coumadin therapy, long term antibiotic tx can all result in this acquired coag disorder:
Vitamin K deficiency
| affecting factors 2, 7, 9, 10, Proteins C,S
136
Heparin binds ___ which greatly enhances ability to bind and inactivate ___:
autoprothrombin 3
| thrombin
137
TT is affected by ___ and ____:
FDP's
| heparin
138
If PT, APTT, and TT are all prolonged, presence of ___ should be considered before factor deficiencies:
heparin
139
How would you test for heparin in sample:
add protamine sulfate to inhibit heparin and normalize prolonged tests
140
Circulating anticoagulants are also called ____:
Inhibitors
141
List the two types of inhibitors:
1) Specific (Ab to specific factor)
| 2) Non-specific (LA- interferes with phospholipid component of reagent)
142
Inhibitors are also called:
Circulating anticoagulants
143
_____ should be suspected in anyone with no prior history who presents with massive bruising or hematoma:
```
Acquired hemophilila
(F8:C inhibition via autoantibodies)
```
144
Autoantibodies to F8:C are most often seen in patients with ___ and has a mortality rate of ___:
RA
SLE
Drug rxns
*~20%
145
Allontibodies to F8:vWF complex are frequently encountered and due to ___:
Transfusion
146
____ should be suspected in any hemophiliac if transfused factor replacement products appear to have reduced effectiveness, hemostasis is hard to achieve, or both:
Inhibitors
147
Factor __ inhibitors are rare, classified as alloAb's, and due to transfusions. Patients do not bleed often, just ___:
F9
| differently (can see hemarthrosis, muscle and soft tissue hemorrhages)
148
In both F8:C and F10, inhibiting antibodies do/do not increase bleeding frequency:
Do not
149
What are the two goals of treating specific inhibitors:
1) stabilize hemostasis
| 2) rid body of antibody
150
In treating specific inhibitors, a ___titer can be treated with high concentrations of Factor concentrates to overwhelm binding sites, while a ___ titer can be treated with steroids, plasmapharesis, cytotoxic treatments, etc:
Low
| High
151
_____ inhibitors are usually accidentally discovered with prolonged APTT screening test, and patient will have increased risk of ___:
Non-specific
| clotting
152
With non-specific inhibitors, prolongation of tests will be see ____, while hypercoagulable state will be seen ____:
in vitro
| in vivo
153
PT and APTT are ___ dependent tests:
phospholipid
154
T/F
| IgG/IgM interefere with phospholipid dependent tests:
True
155
Would mixing studies correct a non-specific inhibitor with NPP:
No. It's not a factor deficiency.
156
APLS stand for:
Antiphospholipid Antibody Syndromes
157
APLS are the most common causes of acquired coag defects associated with thrombosis, list the 4 covered in class:
* aPL (antiphospholipid Ab's)
* aCL (anticardiolipin Ab's)
* LAC/LA (Lupus Anticoagulant)
* CAPS (catastrophic antiphos syndrome)
158
____causes infarction/clots in multiple organs with a ~50% fatality rate:
CAPS
| Catastrophic antiphospholipid syndrome
159
aCL stands for:
anti-cardiolipin
160
LA: autoantibodies react against phospholipid portion of ______:
APTT reagent
161
Is LA more frequently associated with venous or arterial thrombosis:
Venous
162
This develops in 31% of SLE patients, and in those taking psych meds, anti-emetics, antihistamines, and those with lymphoproliferative disorders:
LA
163
Patients with SLE and LA have a 30-40% risk of ____:
Thrombosis
| more likely venous than arterial
164
Which coag test is abnormal with LA:
APTT (PT could also be affected)
| LA autoantibodies react against the phospolipid portion of APTT reagent
165
Would a factor assay be abnormal with LA:
No. It is not a factor deficiency.
166
What test could be done to exclude another coagulopathy that could give similar lab results to that of LA:
Factor assays.
| LA will be normal since it is not a factor deficiency.
167
List the order of factors from intrinsic pathway activation to the fibrin clot:
```
Fletcher (contact)
Fitzgerald (contact)
12
11
9
8
10
5
2
1
13
```
168
List the order of factors from extrinsic pathway activation to the fibrin clot:
```
3 (trauma)
7
10
5
2
1
13
```
169
What converts fibrinogen to fibrin:
Thrombin
170
What converts plasminogen to plasmin:
t-PA
171
____ is triggered by the exposure of negative charge on collagen; Factors Fitzgerald, Fletcher, and 12 all for a complex on the collagen:
Intrinsic pathway
172
____ is the primary and most important pathway, and is triggered by the exposure of F3 in the damaged blood vessel wall to the circulating F7:
Extrinsic pathway
173
List the 3 components in the cascade that utilize negative feedback to enable clot dissolution:
Active Protein C
TFPI
Antithrombin
174
____ works on F10 and Thrombin, the effect is enhanced if heparin is present:
Antithrombin
175
TFPI inhibits conversion of ___ to ___:
7 to 7a
176
If antithrombin is inhibiting Thrombin, what conversion cannot take place:
Fibrinogen to Fibrin
177
Activated Protein C can inhibit the conversion of these two factors to their active forms:
Protein C can inhibit:
8 to 8a
5 to 5a
178
Antithrombin can inhibit the conversion of these two:
10 to 10a
| Fibrinogen to fibrin (by blocking thrombin)
