Haemostasis + inherited bleeding disorders

Question 1

Fuel supply in platelets

Answer 1

Alpha granules and DNA bodies

Question 2

Most abundant glycoprotein molecule receptors on platelets

Answer 2

GpIIb/IIIa heterodimer complex

GpIb

Question 3

Phospholipid in platelet cell membrane

Answer 3

Associated with prostaglandin synthesis, calcium mobilisation, localisation of coagulant activity to the platelet surface

Question 4

Alpha granules

Answer 4

VWF
Platelet factor 4
fibrinogen
Tissue plasminogen activator inhibitor-1

Question 5

Bernard Soulier syndrome

Answer 5

Lack GpIb

Between platelets and VWF

Question 6

Glanzmann’s syndrome

Answer 6

Lack GpIIb/IIIa

Question 7

Treatment for BS + G syndrome

Answer 7

Platelets

Novoseven

Question 8

Von Willebrand disease Type 1

Answer 8

Mild to moderate deficiency of vWF

Question 9

VWD Type 2

Answer 9

vWF protein present but defective

qualitative + quantitative deficiency

Question 10

VWD Type 3

Answer 10

Total absent protein

Question 11

Desmopressin (DDAVP)

Answer 11

Mild cases VWF

Contains tranexamic acid

Question 12

Tissue factor

Answer 12

Present on subendothelial tissue
Expressed by cells which are normally exposed to flowing blood
Physical injury exposes TF to flowing blood

Question 13

Tissue factor function

Answer 13

Necessary for initiation of coagulation

Via factor VII

Question 14

Serum

Answer 14

Plasma without clotting factors

Question 15

Thrombin time measures

Answer 15

fibrinogen –> fibrin

Question 16

Factor 7 problem

Answer 16

PT fucked

Question 17

Factor 8 problem

Answer 17

APTT fucked

Question 18

Haemophilia A

Answer 18

Factor 8 problem

Question 19

Haemophilia B

Answer 19

Factor 9 problem

Question 20

Antithrombin

Answer 20

Major inhibitor of thrombin and Xa
Also inhibits VII, IX and XI
Member of SERine Protease INhibitor family

Question 21

Antithrombin with heparin

Answer 21

Antithrombin inhibitory activity increases 5-10,000 fold in presence of heparin

Question 22

Protein C

Answer 22

Activated by thrombin 
Anti-clotting agent
Form of serine protease
Inactivates Va and VIIIa
Prevents clot from spreading

Question 23

Protein S

Answer 23

Cofactor for Activated Protein C