Secondary Hemostasis Flashcards
1
Q
secondary hemostasis generates (blank) which stabilizes the PLT plug
A
thrombin
2
Q
Thrombin converts fibrinogen to (blank)
A
fibrin
3
Q
The formation of fibrin allows it to be (blanked) to generate strength
A
cross-linked
4
Q
Where are the coagulation factors produced?
A
in the liver
5
Q
T/F: coag factors are produced in an inactive state
A
true
6
Q
What is the activating substance for the extrinsic (activates VII) pathway?
A
tissue thromboplastin (TT)
7
Q
What is the activating substance for the intrinsic (activates XII) pathway?
A
subendothelial collagen (SEC)
8
Q
What are the three things needed to activate the coag cascade?
A
- activating factor (SEC or TT)
- phospholipid surface of PLTs
- Calcium from PLT dense granules
9
Q
What distinguishes 2ry hemostatic disease from 1ry?
A
2ry causes deept tissue bleeding into muscles and joints (hemarthrosis) and rebleeding after surgical procedures (circumcision and tooth extraction common)
10
Q
What factors are in the intrinsic pathway?
A
12, 11, 9, 8
11
Q
What factors are in the extrinsic (tissue factor) pathway?
A
7; 7 is lucky, luck is extrinsic
12
Q
What factors are in the common pathway?
A
5, 2, 1
13
Q
What factors are activated by Vit. K?
A
2, 7, 9, 10, C, S
14
Q
(PT/PTT) measures the extrinsic pathway (7)
A
PT; TT activates 7, PT and TT both have 2 letters
15
Q
(PT/PTT) measures the intrinsic pathway (12)
A
PTT; SEC activates 12, PTT and SEC both have 3 letters
16
Q
Hemophilia A is caused by a def of what factor?
A
AAAAAAAAA-8!
17
Q
What is the mode of inheritance in males of hemophilia A?
A
x-linked
18
Q
T/F: hemophilia A can present with no family history
A
true
19
Q
What is the most common presentation of hemophilia a?
A
deep tissue bleeding after surgery
20
Q
T/F: severity of hemophilia A is in a range depending on how def you are
A
true
21
Q
What are the lab values for hemophilia a? PT/PTT factor levels PLT bleeding time
A
HI PTT normal PT low FVIII NORMAL PLT normal bleeding time (if superficial can use primary hemostasis)
22
Q
How do you treat hemophilia A?
A
recombinant FVIII
23
Q
What is Christmas disease ?
A
hemophilia B, factor 9 def
24
Q
What is the only difference between hemophilia A and B?
A
B has a 9 def
25
T/F: a mixing study will normalize the PTT in both hemophilia a and B
true
26
T/F: a mixing study will normalize PTTT in a coagulation factor inhibitor
FALSE; factor will overwhelm the mixed serum
27
What is the most common anti-factor inhibitor?
anti-8
28
T/F: inhibitor disorders can cause qualitative and quantitative disorders
true
29
What is the most common INHERITED coag disorder?
vW disease
30
How does vW disease normally present?
mild skin and mucosal bleeding
31
vW disease is due to impaired PLT (adhesion/aggregation)
adhesion
32
```
Describe the lab values for vW disease
bleeding time
PT/PTT
PLT
Ristocetin test
```
increased bleeding time
increased PTT: decreased FV8 b/c vWF stabilizes 8
normal PT
abnormal ristocetin leading to NO agglutination
33
What is the Tx for vW disease?
desmopressin
34
Desmopressin increases vWF release from the (blank) bodies of endothelial cells
weibel-palade
35
Vit. K is activated by (blank) in the liver
epoxide reductase
36
Vit. K gamma carboxylates what factors?
2 7 9 10 C S
37
PT is used to test (heparin/coumadin) levels
PT = coumadin/warfrin
| two letters, two drug names
38
PTT is used to test (heparin/coumadin)
PTT = heparin; HEParin has three letters like PTT
39
Coumadin blocks (blank) activity resulting in less activated Vit. K
epoxide reductase
40
what are the three scenarios in which you see a Vit. K def?
1. newborns --lack of GI bacteria to make vit. K
2. long term abx therapy
3. malabsorption
41
How does liver failure lead to problems with the coag cascade?
Liver is the source of all the factors AND of Vit. K activation.
DECREASED PRODUCTION
DECREASED ACTIVATION
42
How does a large volume transfusion mimmic a coag cascade def?
Works to dilute the factors
43
WHat is another name for factor I?
fibrinogen
44
What is another name for prothrombin?
factor II
45
What coag factor is made by the Kuppfer cells in the liver?
FVIII
46
WHere is Prekalikrein made?
liver
47
Where is high molecular weight kininogen made?
liver
48
What is another name for factor XIII
fibrin stabilizing factor
49
What is Hagemann factor?
FXII
50
What is christmas factor
FIX
51
What is antihemophilic factor?
FVIII
52
What two factors make up the prothrombinase complex?
Va and Xa
53
What three factors make up the tenase complex?
VIIIa, Xa, and Ca
54
What two factors are needed to convert XII to XIIa?
prekallikrein and high molecular weight kininogen
55
What ion is needed to transform VIIIa to X?
Ca
56
What factor is needed to take cross link fibrin?
XIIIa
57
What factor is needed to take Fibrinogen to fibrin?
IIa
58
Plasmin degrades what end product of the coag cascade?
fibrin
59
what coag path is activated by tissue injury?
Extrinisc via 7
60
Which coag path is activated by contact?
Intrinsic path
61
What test is done by adding phospholipid (contact activator) to calcium and plasma?
PTT
62
What test is done by adding tissue factor and phospholipid to plasma?
PT
63
What test screens for qualitative PLT disorders?
bleeding time
64
What disease presents with this PENTAD of symptoms:
1. thrombocytopenia
2. hemolytic anemia
3. neurologic abn.
4. renal abn.
5. fever
TTP
65
what is the mode of inheritance of activated protein C resistance?
Auto Dom
66
(blank) is responsible for 50% of hereditary thrombophilia
activated protein C resistance
67
What is the MOA of activated protein C resistance? (think Factor V)
heterozygosity of FV Leidin which makes FVL resistance to inactivation via protein C
68
An anthithrombin deficiency leads to no inactivation of what factors?
II, IXa, Xa, XIa, XIIIa
| 2 9a 10a 11a 12a
69
what is the MOA of anthithrombin?
auto dom
70
Protein C is activated by the (blank) complex
thrombin-thrombomodulin complex
71
What is the cofactor for the thrombin-thrombomodulin complex?
protein S
72
Acquired (blank) deficiency may be due to coumadin therapy, liver disease, and pregnancy
protein C
73
Antiphospholipid syndrome includes what two antibodies?
lupus anticoagulant syndrome
| anti-cardiolipin syndrome
74
In what diseases do you seen antiphospholipid syndrome?
lupus, primary APL syndrome, HIV, malignancy, collagen vascular disorders, and with drugs
75
What is the second most common causes of inherited thrombophilia?
prothrombin variant
76
thrombin can act on what activated factors?
2a 9a 10a 11a 12a
77
Is the protein C receptor antithrombotic or prothrombotic?
anithrombotic
78
Is tPA antithrombotic or prothrombotic?
antithrombotic
79
Is Thromboxane antithrombotic or prothrombotic?
prothrombotic
80
Are NO and prostacyclin antithrombotic or prothrombotic?
anithrombotic
81
What platelet receptor binds vWF?
GP1b
82
What platelet receptor binds fibrinogen?
GpIIb/IIIa
83
What platelet receptor binds fibronectin?
GPIc/IIa
84
What platelet receptor is an adhesion molecule?
CD62P (P-selectin)
85
What platelet receptor binds initiates platelet activation when bound by thrombin?
thrombin receptor
86
What platelet receptor binds initiates PLT activation when bound by ADP?
ADP receptor
87
What platelet receptor binds collagen?
GPIa/IIa
88
T/F: PLTs contain red cell antigens
true
89
T/F: PLTs contain Rh factor
FALSE
90
What class of MHC ag is present on PLTs?
MHC I
91
What particular MHCs are present on PLTs?
HLA-A, B, and C
92
What is contained within the dense granules of PLTs?
ATP
ADP
Ca
Serotonin
93
What is contained within the a-granules of PLTs?
```
vWF
Fibrinogen
FV
VEGF, EGF, PDGF
Angiostatin, thrombospondin, endostatin
PF-4, IL-8, CCL5
```
94
PGI2 released by endothelial cells has a (pos/neg) effect on the coag cascade
negative
95
Tissue Factor Pathway Inhibitor blocks what conversion?
7 to 7a
96
tPA prevents what final step of stabilizing the PLT plug?
cross linking of fibrin;
| tpA converts plasminogen to plasmin, and plasmin blocks the cross linking
97
What test do you use to screen for vW disease?
bleeding time
98
T/F: bleeding time is useful to predict the risk of bleeding during surgery
false
99
What test uses ADP, epi, arachidonate, collagen, and ristocetin to determin PLT aggregation?
PLT aggregometry
100
What is PLT flow cytometry used to Dx?
def. of PLT surface glycoproteins and disorders of PLT activation
101
INR is used to monitor (heparin/coumadin) therapy
coumadin
102
INR references the (PT/PTT)
PT
103
What test is used to monitor heparin, hirudin, or argatroban therapy?
aPTT
104
What test is used to test the presence of functional fibrinogen?
TT
105
What causes prolongation of the thrombin time?
heparin, hirudin, argatroban and dysfribrinogenemia
106
What does a mixing study screen for?
presence of inhibitors
107
T/F: mixing studies excludes the presence of heparin
true
108
If there is at least 50% activity on the mixing study, then you know that the prolongation is due to (blank)
a factor deficiency
109
If there is less than 50% activity in the mixing study, then you know that the prolongation is due to (blank)
presence of an inhibitor
110
D-dimers are formed by the degradation of what molecule in the clot?
fibrin
111
The fuck do you use an Anti-Xa assay for?
monitor unfractionated or LMWH therapy
112
Males tend to have (coag/PLT) bleeding disorders
coag
113
females tend to have (coag/PLT) bleeding disorders
PLT
114
Type I vW disease is (quantitative/qualitative) disorder
quantitative; not enough vWF
115
```
Describe the lab values for Type I vW disease:
PT
PTT
BT (bleeding time)
FVIII levels
```
normal PT
increased PTT
increased bleeding time
decreased FVIII
116
Type II vW disease is (quantitative/qualitative)
qualitative
117
Type IIM vW prevents vWF from binding to (blank)
GpIb
118
Type IIN vW is shows reduced binding of vWF to (blank)
FVIII
119
Type IIa vW is absence of (blank)
high molecular wt. vWF multimers
120
What is the only lab value that is markedly changed in vWF IIa?
ristocetin agglutination decreased
121
vW disease IIb is spontaneous binding of vWF to (blank)
PLTs
122
T/F: vW IIa and IIb both have decreased high molecular wt. vWF multimers
true
123
What should you not give to someone with vW IIb?
DDAVP: profound thrombocytopenia and bleeding
124
What is wonky about the ristocetin test in vW IIb>?
it is increased since there is spontaneous PLT binding
125
In what disease do PLTs lack the PLA1 ag?
Glanzmann thrombasthenia
126
IN what disorder to PLTs fail to aggregate with all agonists except risocetin?
Glanzmann thrombasthenia
127
Hermansky-Pudlak
Chediak-Higashi
Wiskott-Aldrich
Are all (blank) diseases with no second wave of aggregation
dense granule
128
In alpha granule disease, aggregation is blunted with all agents except (blank)
ADP
129
Gray PLT syndrome is a (a/dense) granule disorder
alpha
130
ASA and NSAIDS inhibit what enzyme?
COX-1
131
What molecule released by first wave PLTs from the dense granules signal for the second wave aggregation?
tXA2
132
Is the effect of ASA or NSAIDS on COX-1 irreversible?
ASA
133
Ticlopidine and Clopidogrel inhibit (blank) mediated PLT activation
ADP
134
Abciximab, Eptifibatide, Tirofiban inhibit the (blank) receptor, aka fibrinogen receptor
GpIIb/IIIa
135
Abciximab, Eptifibatide, Tirofiban will cause impaired aggregation to which agonists?
all except ristocetin
136
Ticlopidine & Clopidogrel will cause impaired aggregation against which agonist?
ADP
137
What bleeding disease is made by a diagnosis of exclusion?
ITP
138
Heparin induced thrombocytopenia is IgG binding to what complex?
Heparin-PF4 between the drug and the PLT
139
T/F: bleeding is rare in HIT
true
140
T/F: thrombosis is likely in HIT
true, as strange as it is considering the name; PLTs will aggregate and activate the fucking cascade
141
Liver disease results in chronic DIC from an impaired clearance of (blank)
d-dimer
142
anti-thrombin def. results in (arterial/venous) thromboses
venous
143
T/F: homozygous anti-thrombin def is incompatible with life
true
144
antibodies against B2 glycoproteins and prothrombin are most likely what type of Ab?
antiphospholipid; can be classified as lupus anticoagulant or anticardiolipin
145
phenytoin, quinidine, hydralazine, procainamide, phenothiazines, IFN, cocaine
can all cause secondary (blank)
antiphospholipid syndrome
146
Coumadin therapy may result in an acquired (blank and blank) def
protein C and S
147
Ca is necessary in the conversion of what factor from the intrinsic pathway to the common pathway?
8a to X
