Exam 2 Material

Question 1

Coagulation proteins in circulation that are inactive are called:

Answer 1

zymogens

Question 2

What is the purpose of activated coagulation proteins:

Answer 2

interact to form fibrin clots, reinforcing the platelet plug

Question 3

How were the coag proteins named:

Answer 3

based on sequence of discovery

Question 4

All coag factors are produced in the ___:

Answer 4

liver

Question 5

Factor 1:

Answer 5

Fibrinogen

Question 6

Fibrinogen is Factor___:

Answer 6

1

Question 7

Factor 2:

Answer 7

Prothrombin

Question 8

Prothrombin is Factor__:

Answer 8

2

Question 9

Tissue Factor is factor ___:

Answer 9

3

Question 10

Factor 3 is ____:

Answer 10

Tissue Factor

Question 11

Ionized calcium is factor___:

Answer 11

4

Question 12

Factor 4 is:

Answer 12

Ionized calcium

Question 13

Labile Factor is factor___:

Answer 13

5

Question 14

Factor 5 is ____:

Answer 14

Labile Factor

Question 15

Stable factor/proconvertin is factor___:

Answer 15

7

Question 16

Factor 7 is ___:

Answer 16

Stable factor/proconvertin

Question 17

Antihemophilic factor is factor___:

Answer 17

8

Question 18

Factor 8 is ___:

Answer 18

Antihemophilic factor

Question 19

Christmas factor is factor___:

Answer 19

9

Question 20

Factor 9 is _____:

Answer 20

Christmas factor

Question 21

Stuart-Prower factor is factor ___:

Answer 21

10

Question 22

Factor 10 is ____:

Answer 22

Stuart Prower Factor

Question 23

PTA is factor___:

Plasma Thromboplastin Antecedent

Answer 23

11

Question 24

Factor 11 is ___:

Answer 24

PTA

Plasma Thromboplastin Antecedent

Question 25

Hageman Factor is factor ___:

Answer 25

12

Question 26

Factor 12 is ___:

Answer 26

Hageman Factor

Question 27

FSF is factor___:

Fibrin Stabilizing Factor

Answer 27

13

Question 28

Factor 13 is ____:

Answer 28

FSF

Fibrin Stabilizing Factor

Question 29

Fitzgerald Factor is ______:

Answer 29

HMWK

Question 30

HMWK is _____ factor:

Answer 30

Fitzgerald Factor

Question 31

Fletcher factor is ____:

Answer 31

Prekallikrein

Question 32

Prekallikrein is ____ factor:

Answer 32

Fletcher

Question 33

What is the main substrate in the coag cascade:

Answer 33

fibrinogen

Question 34

List the two cofactors of the coag cascade:

Answer 34

• Factor 5

* Factor 8:C

Question 35

What do serine proteases do:

Answer 35

cut peptide bonds

Question 36

Serine proteases include all but which factor:

Answer 36

Factor 8

Question 37

_____ create cross linking in fibrin clot:

Answer 37

transaminases

Question 38

_______ cut peptide bonds:

Answer 38

Serine proteases

Question 39

Factor 8a is the only _____:

Answer 39

Transaminase

Question 40

Contact proteins are only involved in initial phase of intrinsic activation and not consumed in normal clotting process, list the 4 of them:

Answer 40

11
12
Fitzgerald
Fletcher

Question 41

Coumadin works ____, while heparin works _____:

Answer 41

Coumadin: in vivo
Heparin: in vivo and in vitro

Question 42

List the Vit K dependent factors:

Answer 42

2,7, 9, 10

Protein C & S

Question 43

PT is used to monitor ____ therapy:

Answer 43

Coumadin/Warfarin

Question 44

Acquired Vit K deficiences most commonly seen in the following:

Answer 44

• post-surgical
• High dose antibiotic use
• liver disease
• malnutrition

Question 45

List the fibrinogen or thrombin sensitive proteins (i.e. thrombin acts upon them):

Answer 45

1, 5, 8:C, 13

Question 46

____ acts on factors 1, 5, 8:C, and 13:

Answer 46

Thrombin

Question 47

_____ acts on all factors of in the fibrinogen group:

Answer 47

Thrombin

Question 48

Which factors have positive feedback, procoagulant effects with Thrombin:

Answer 48

Factors 5 and 8

Question 49

How is Factor 1 converted to soluble fibrin monomer:

Answer 49

Fibrinopeptides A & B are cleaved

Question 50

Coag inhibitor binds to antithrombin (AT), and presence of heparin enhances this binding by how much:

Answer 50

200 times

Question 51

______ has procoagulant and coagulation inhibiting effects:

Answer 51

Thrombin

Question 52

The procoagulant effects of Thrombin rely on ___ feedback with Factors 5 and 8:

Answer 52

positive

Question 53

The coagulation inhibiting effects of Thrombin binds to Thrombomodulin, via ___feedback with Factors 5a and 8a:

Answer 53

negative feedback

keeps clotting from getting out of control

Question 54

Thrombin initiates tissue repair via these two mechanisms:

Answer 54

• induces chemotaxis

* stimulates proliferation of smooth muscle and endothelial cells

Question 55

Which anticoagulants affect the extrinsic pathway:

Answer 55

coumadin/warfarin

test via PT

Question 56

Why must calcium be added to a specimen to activate the coag cascade:

Answer 56

The citrate in a light blue top tube chelates the specimen calcium, so more must be added

Question 57

Excess of PAI-1 is associated with _____:

Answer 57

thrombotic disease

higher in MI patients >45

Question 58

The primary substrate of PAI-1 is ___, thus regulation of fibrinolysis is dependent on the interaction of ___ with ____:

Answer 58

t-PA

t-PA with PAI-1

Question 59

T/F

PAI-1 is an acute phase reactant that is synthesized by blood vessel endothelium and released in an inactive state:

Answer 59

True.

Question 60

BT would be ____ in fibrinogen disorders because ____:

Answer 60

normal

BT tests platelet function

Question 61

_____ is a quantitative disorder causes by lack of synthesis in the liver:

Answer 61

afibrinogenemia

Question 62

Levels of fibrinogen <100, generally asymptomatic:

Answer 62

hypofibrinogenemia

Question 63

Abnormal structure and function of fibrinogen; qualitative disorder:

Answer 63

Dysfibrinogenemia

Question 64

In dysfibrinogenemia, would BT and fibrinogen levels likely be normal:

Answer 64

Yes.

Question 65

____ functions as a catalyst, forming bonds between proteins (fibrin monomers, fibronectin, collagen, alpha 2 inhibitor):

Answer 65

F13

Question 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 ____:

Answer 66

Factor 13

5M urea test

Question 67

List the 3 acquired disorders of secondary hemostasis:

Answer 67

• DIC
• Primary Fibrinolysis
• Liver disease

Question 68

Why will the PT/APTT/ and TT be prolonged in DIC:

Answer 68

Due to consumption coagulopathy

Question 69

What is the first and foremost treatment of DIC:

Answer 69

Remove stimulus!
then:
*LMWH, FFP, cryo

Question 70

____ results from increased levels of plasmin:

Answer 70

Primary Fibrinolysis

Question 71

What 3 things are normal in Fibrinolysis, but abnormal in DIC:

Answer 71

• d-dimer (high in DIC)
• Plt count (low in DIC)
• RBC’s (fragments in DIC)

Question 72

Factor ___ has the shortest half-life:

Answer 72

7

Question 73

You’d see a decrease in ___ factors in mild liver disease:

Answer 73

vit k dependent factors

Question 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:

Answer 74

liver disease

Question 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:

Answer 75

Primary Fibrinolysis

Question 76

Pregancy, cancer metastases, promyelocytic leukemia would be____ causes of DIC:

Answer 76

Extrinsic

Question 77

Events that damage vascular endothelium and expose collagen such as infectious diseases, snake venom, massive trauma/surgery would be ____ causes of DIC:

Answer 77

Intrinsic

Question 78

_____ is seen in 1/ of chronic liver disease patients due to spleen sequestration secondary to congestive splenomegaly:

Answer 78

Thrombocytopenia

Question 79

Congjugated estrogen can be used as a treatment for thrombocytopenia caused by ____:

Answer 79

liver disease

Question 80

Hemophilia A is a deficiency of ____, and is the most common hereditary coag disorder:

Answer 80

Factor 8:C

Question 81

Hemophilia A exhibits this type of inheritance:

Answer 81

X-linked recessive

Question 82

Severe hemophilia A has F8:C levels of ____ and requires constant transfusion therapy of ___:

Answer 82

<1%

Factor 8 concentrates

Question 83

Why is there an increased risk of developing alloantibodies or inhibitors to F8:C in Hemophilia A:

Answer 83

Due to regular F8:C infusions

Question 84

Symptoms of alloantibodies to F8:C include:

Answer 84

hemarthrosis
hematuria
intracranial bleeds
hematomas

Question 85

Why is a recombinant factor replacement preferred:

Answer 85

Risk of alloantibodies

Question 86

In Hemophilia A, mixing studies do not correct with ____, which means either an inhibitor is present or reacting antibodies:

Answer 86

normal pool plasma

Question 87

Which coag test will be abnorml in Hemophilila A:

Answer 87

APTT

prolonged if F8<20%

Question 88

Also known as Christmas Disease:

Answer 88

Hemophilia B (F9 deficiency)

Question 89

Hemophilia B is a deficiency of factor__:

Answer 89

9

Question 90

Factor 9 can be activated by ___:

Answer 90

11a+Ca, or Russell Viper Venom

Question 91

T/F

Hemophilia B is sex-linked and less common than Hemophilia A:

Answer 91

True

Question 92

Which has a longer half-life, F8:C or F9 concentrates:

Answer 92

F9: 24 hrs
F8:C 12 hrs

Question 93

Can Hemophilia B be acquired, and if so, how:

Answer 93

Yes.

Liver disease, Vit K deficiency, oral antiocoagulant therapy

Question 94

List the respective deficiency of each Hemophilia:
A:
B:
C:

Answer 94

A: 8:C
B: 9
C: 11

Question 95

Which coag test would be abnormal in Hemophilia B, and would mixing studies correct with NPP and AP:

Answer 95

APTT (F9 is intrinsic)

Yes

Question 96

Aged serum contains which factors:

Answer 96

7
9
10
11
12

Question 97

Adsorbed plasma has which factors removed:

Answer 97

Vit K dependent

Question 98

Adsorbed plasma contains which factors:

Answer 98

1
5
8
11
12

Question 99

Hemophilia C is also known as ____:

Answer 99

Rosenthal Syndrome

Question 100

Rosenthal syndrome is also known as ______ and is predominantly in the ____ population:

Answer 100

Hemophilia C

Ashkenazi Jewish population (1:8)

Question 101

Bleeding after dental surgery/extraction in an Ashkenazi Jew, think ____:

Answer 101

Hemophilia C

Question 102

Why is F11 replacement not needed in Hemophilia C unless patient is scheduled for surgery:

Answer 102

F11 is early in the cascade, and there are other ways it can be initiated

Question 103

__or ____ possible in severe F11 deficiency:

Answer 103

alloantibodies or inhibitors

Question 104

F11 levels >120% increase risk of ___:

Answer 104

thrombosis

Question 105

Overabundance of F11 will bind to ___ binding sites, preventing ____ from activating, therefore preventing fibrinolysis, resulting in ____:

Answer 105

plasminogen
plasminogen
thrombosis

Question 106

Which coag test will be abnormal in Hemophilia C:

Answer 106

APTT

Question 107

vWD occurs almost as frequently as ____:

Answer 107

Hemophilia A

Question 108

Will the BT be increased or decreased in vWD:

Answer 108

increased

Question 109

Plt aggregation in vWD is impaired with ___:

Answer 109

Ristocetin

Question 110

Factor 7 deficiency (aka Proconvertin) has symptoms similar to factor ___ deficiency:

Answer 110

8

Question 111

Deficiency of factor___ will have a prolonged PT that will fully correct with RVV and mixing with aged serum:

Answer 111

Factor 7 deficiency

Question 112

How does RVV correct a Factor 7 deficiency:

Answer 112

It activates Factor 10

Question 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:

Answer 113

True.

Question 114

Is Stypven Time prolonged in Factor 10 deficiency:

Answer 114

Yes. (depends on F2, 5, 10 w/ phospholipid)

Question 115

With the Factor 10 assay, variant forms of F10 could show ____ between tests:

Answer 115

discrepancies

Question 116

___activity of F10 is considered adequate for hemostasis:

Answer 116

10%

Question 117

Factor 5 deficiency is also known as ___:

Answer 117

parahemophilia

Question 118

Factor 5 is also called _____ because of rapid deterioration in plasma at room temp:

Answer 118

labile

Question 119

F5 is a catalyst in conversion of ___to ___:

Answer 119

F2–>F2a

Question 120

Acquired F5 deficiency can occur from specific Ab acquired after ____ or use of ___ in surgery:

Answer 120

childbirth

fibrin glue

Question 121

1/2 of F5 deficient patients have an increased __ due to plt-related function of F5 of binding ___ to plt surface:

Answer 121

BT

F10

Question 122

In testing for a F5 deficiency, specimens must be ____:

Answer 122

platelet poor (<10k)

Question 123

Deficiency of this delays generation of thrombin, causing severe hemorrhagic symptoms:

Answer 123

Factor 2 (Prothrombin)

Question 124

The ‘Prothrombin Complex’ includes everything from ___ to ___:

Answer 124

F2 to Thrombin

Question 125

Hypoprothrombinemia is a deficiency of this factor:

Answer 125

F2 (prothrombin)

Question 126

Dysprothrombinemia is a structural defect of ___ that causes _____:

Answer 126

F2 (prothrombin)

impaired activity

Question 127

A single point mutation on chromosome 11 leads to a mutation of factor___ and is the 2nd most common cause of _____:

Answer 127

F2 (prothrombin)

inherited thrombophilia

Question 128

Does the Prothrombin Mutation on Chromosome 11 lead to an increased risk of clotting or bleeding:

Answer 128

clot risk

• risk factor for MI/stroke in young pts
• mostly caucasian

Question 129

____deficiency is not associated with clinical bleeding or hemorrhage, patients are asymptomatic and post no surgical risk, even though their APTT is prolonged:

Answer 129

F12 (Hageman factor)

Question 130

Deficiencies of Fletcher or Fitzgerald factors will both show marked prolongation of ____:

Answer 130

APTT

Question 131

In Fletcher deficiency, contact activation time with APTT kaolin-like reagents changes with incubation time intervals, the APTT intervals will progessively ___:

Answer 131

shorten

Question 132

Is there any racial predilection or apparent clinical bleeding associated with Fletcher or Fitzgerald factor deficiencies:

Answer 132

No.

Question 133

One could acquire inhibition of factor____ following TB treatment with Isoniazid:

Answer 133

F13

Question 134

Autoimmune inhibitors of F8:C most commonly result from___ and produce ___class of antibodies:

Answer 134

F8:C transfusions
IgG
(can also be seen in patients with RA, SLE, drug rx, post partum, etc)

Question 135

Poor diet, biliary obstruction, malabsorption, coumadin therapy, long term antibiotic tx can all result in this acquired coag disorder:

Answer 135

Vitamin K deficiency

affecting factors 2, 7, 9, 10, Proteins C,S

Question 136

Heparin binds ___ which greatly enhances ability to bind and inactivate ___:

Answer 136

autoprothrombin 3

thrombin

Question 137

TT is affected by ___ and ____:

Answer 137

FDP’s

heparin

Question 138

If PT, APTT, and TT are all prolonged, presence of ___ should be considered before factor deficiencies:

Answer 138

heparin

Question 139

How would you test for heparin in sample:

Answer 139

add protamine sulfate to inhibit heparin and normalize prolonged tests

Question 140

Circulating anticoagulants are also called ____:

Answer 140

Inhibitors

Question 141

List the two types of inhibitors:

Answer 141

1) Specific (Ab to specific factor)

2) Non-specific (LA- interferes with phospholipid component of reagent)

Question 142

Inhibitors are also called:

Answer 142

Circulating anticoagulants

Question 143

_____ should be suspected in anyone with no prior history who presents with massive bruising or hematoma:

Answer 143

Acquired hemophilila
(F8:C inhibition via autoantibodies)

Question 144

Autoantibodies to F8:C are most often seen in patients with ___ and has a mortality rate of ___:

Answer 144

RA
SLE
Drug rxns
*~20%

Question 145

Allontibodies to F8:vWF complex are frequently encountered and due to ___:

Answer 145

Transfusion

Question 146

____ should be suspected in any hemophiliac if transfused factor replacement products appear to have reduced effectiveness, hemostasis is hard to achieve, or both:

Answer 146

Inhibitors

Question 147

Factor __ inhibitors are rare, classified as alloAb’s, and due to transfusions. Patients do not bleed often, just ___:

Answer 147

F9

differently (can see hemarthrosis, muscle and soft tissue hemorrhages)

Question 148

In both F8:C and F10, inhibiting antibodies do/do not increase bleeding frequency:

Answer 148

Do not

Question 149

What are the two goals of treating specific inhibitors:

Answer 149

1) stabilize hemostasis

2) rid body of antibody

Question 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:

Answer 150

Low

High

Question 151

_____ inhibitors are usually accidentally discovered with prolonged APTT screening test, and patient will have increased risk of ___:

Answer 151

Non-specific

clotting

Question 152

With non-specific inhibitors, prolongation of tests will be see ____, while hypercoagulable state will be seen ____:

Answer 152

in vitro

in vivo

Question 153

PT and APTT are ___ dependent tests:

Answer 153

phospholipid

Question 154

T/F

IgG/IgM interefere with phospholipid dependent tests:

Answer 154

True

Question 155

Would mixing studies correct a non-specific inhibitor with NPP:

Answer 155

No. It’s not a factor deficiency.

Question 156

APLS stand for:

Answer 156

Antiphospholipid Antibody Syndromes

Question 157

APLS are the most common causes of acquired coag defects associated with thrombosis, list the 4 covered in class:

Answer 157

• aPL (antiphospholipid Ab’s)
• aCL (anticardiolipin Ab’s)
• LAC/LA (Lupus Anticoagulant)
• CAPS (catastrophic antiphos syndrome)

Question 158

____causes infarction/clots in multiple organs with a ~50% fatality rate:

Answer 158

CAPS

Catastrophic antiphospholipid syndrome

Question 159

aCL stands for:

Answer 159

anti-cardiolipin

Question 160

LA: autoantibodies react against phospholipid portion of ______:

Answer 160

APTT reagent

Question 161

Is LA more frequently associated with venous or arterial thrombosis:

Answer 161

Venous

Question 162

This develops in 31% of SLE patients, and in those taking psych meds, anti-emetics, antihistamines, and those with lymphoproliferative disorders:

Answer 162

LA

Question 163

Patients with SLE and LA have a 30-40% risk of ____:

Answer 163

Thrombosis

more likely venous than arterial

Question 164

Which coag test is abnormal with LA:

Answer 164

APTT (PT could also be affected)

LA autoantibodies react against the phospolipid portion of APTT reagent

Question 165

Would a factor assay be abnormal with LA:

Answer 165

No. It is not a factor deficiency.

Question 166

What test could be done to exclude another coagulopathy that could give similar lab results to that of LA:

Answer 166

Factor assays.

LA will be normal since it is not a factor deficiency.

Question 167

List the order of factors from intrinsic pathway activation to the fibrin clot:

Answer 167

Fletcher (contact)
Fitzgerald (contact)
12
11
9
8
10
5
2
1
13

Question 168

List the order of factors from extrinsic pathway activation to the fibrin clot:

Answer 168

3 (trauma)
7
10
5
2
1
13

Question 169

What converts fibrinogen to fibrin:

Answer 169

Thrombin

Question 170

What converts plasminogen to plasmin:

Answer 170

t-PA

Question 171

____ is triggered by the exposure of negative charge on collagen; Factors Fitzgerald, Fletcher, and 12 all for a complex on the collagen:

Answer 171

Intrinsic pathway

Question 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:

Answer 172

Extrinsic pathway

Question 173

List the 3 components in the cascade that utilize negative feedback to enable clot dissolution:

Answer 173

Active Protein C
TFPI
Antithrombin

Question 174

____ works on F10 and Thrombin, the effect is enhanced if heparin is present:

Answer 174

Antithrombin

Question 175

TFPI inhibits conversion of ___ to ___:

Answer 175

7 to 7a

Question 176

If antithrombin is inhibiting Thrombin, what conversion cannot take place:

Answer 176

Fibrinogen to Fibrin

Question 177

Activated Protein C can inhibit the conversion of these two factors to their active forms:

Answer 177

Protein C can inhibit:
8 to 8a
5 to 5a

Question 178

Antithrombin can inhibit the conversion of these two:

Answer 178

10 to 10a

Fibrinogen to fibrin (by blocking thrombin)