Coagulation Details

Question 1

Glanzmann Thrombasthenia is a deficiency of…?

Answer 1

GP IIb/IIIa

Question 2

Bernard Soulier Syndrome is a deficiency of…?

Answer 2

GP Ib

Question 3

Activated protein C mediates what?

Answer 3

Degradation of Factor 5a and 8a

Question 4

What signals upregulate GP IIb/IIIa to cause platelet aggregation?

Answer 4

ADP

Question 5

Factors 2, 7, 9, 10 are all produced where? What enzyme is required for their synthesis?

Answer 5

Liver

Epoxide reductase

Question 6

Fletcher factor is AKA…

Answer 6

Prekallikrein

Question 7

Fitzgerald Factor is AKA…

Answer 7

HMW Kininogen

Question 8

Virchow’s Triad

Answer 8

Hypercoagulable State
Abnormal Blood Flow
Endothelial Injury

Question 9

Describe basic mechanism of heparin induced thrombocytopenia

Answer 9

Heparin is administered IV and binds with platelet factor 4 in the bloodstream. Antibodies are generated against this substance, which also have activity against platelets. This causes thrombocytopenia

Question 10

What is the major adverse consequence of heparin induced thrombocytopenia?

Answer 10

Venous limb gangrene

Question 11

Describe the basic mechanism of ITP

Answer 11

Immune Thrombocytopenic Purpura

IgG antibodies against platelets are generated, leading to thrombotic disorders and excess clotting

Question 12

Describe the basic mechanism of TTP

Answer 12

Thrombotic Thrombocytopenic Purpura
Deficiency of an enzyme (ADAMST13) that cleaves vWF multimers leads to excess vWF in the bloodstream. Causes aggregation of platelets without endothelial injury. Aggregates will block the microvasculature, causing microangiopathic hemolytic anemia.

Question 13

What is poikocytosis?

Answer 13

Heterogeneity in cell structure due to the high force by which blood cells are forced through occluded vessels in microangiopathic syndromes

Question 14

What conversion does TFPI specifically inhibit?

Answer 14

Conversion of Factor X to Factor Xa

Question 15

Describe the basic mechanism of Factor V Leiden disease

Answer 15

Mutation leading to Factor V resistance to Protein C degradation. Protein C cannot degrade Factor 5, so these patients have hypercoagulability

Question 16

What does thrombomodulin do?

Answer 16

Activates Protein C to break down Factors 5a and 8a. Therefore, thrombomodulin promotes a hypocoagulable state.

Question 17

Hemophilia A is a deficiency of…?

Answer 17

Factor 8

Question 18

Hemophilia B is a deficiency of…?

Answer 18

Factor 9

Question 19

Describe how you might test for von Willebrand disease

Answer 19

Add ristocetin, which should complex with vWF to cause coagulation. If there is no vWF, the ristocetin will not promote coagulation.

Question 20

Describe the 3 different types of von Willebrand disease

Answer 20

Type 1 and Type 3 = decreased circulating level of vWF (quantitative)

Type 2 = qualitative defect in vWF protein

Question 21

Describe some clinical findings in Hemophilias

Answer 21

Prolonged APTT
Platelet function is normal
Bleeding time is normal

Question 22

Describe some clinical findings in von Willebrand Disease

Answer 22

Impaired adhesion of platelets in vivo.
Bleeding time elevated
APTT slightly elevated

Question 23

What si the main difference between primary and secondary fibrinolysis?

Answer 23

Primary fibrinolysis involves plasmin degrading only fibrinogen.
Secondary fibrinolysis involves plasmin degrading fibrinogen and fibrin.

Question 24

Hyperhomocysteinemia can lead to…

Answer 24

Excess thrombosis