CPT Haem Revision

Question 1

What do platelets release on activation?

Answer 1

ADP
TXA
Serotonin
Calcium
Change shape = express GpIIb/IIIa

Question 2

5-HT function

Answer 2

Platelet chemotaxis

Release by active platlets

Question 3

TXA/ADP function

Answer 3

Activates other platelets

Question 4

Platelet diameter

Answer 4

1-4 micrometer

Question 5

Platelet lifespan

Answer 5

8-14 days

Question 6

Primary Haemostasis

Answer 6

Injury to vessel wall
Vasoconstriction
Exposed collagen
VWF binds to collagen
VWF binds to platelets via Gp1b
Platelet activation = express GpIIb/IIIa
Fibrinogen binds to these receptors
Further platelets bind to fibrinogen

Question 7

Bernard Soulier Syndrome

Answer 7

Lack of Gp1b

Question 8

Glanzmanns syndrome

Answer 8

Lack of GpIIb/IIIa

Question 9

Treatment for Bernard Soulier and Glanzmanns

Answer 9

Platelets or novoseven (recombinant factor VIIa) or transxamic acid

Question 10

VWD types

Answer 10

Mild = mild to moderate deficiency
Moderate = present but defective
Severe = absent

Question 11

VWD Inheritance

Answer 11

Autosomal inheritance

Question 12

VWD Treatment

Answer 12

DDAVP if mild
Tranexamic acid
VWF containing concentrate

Question 13

Intrinsic Pathway

Answer 13

12->12a converts 11->11a converts 9->9a.
9-> 9a requires XIa and calcium
9a converts 10-> 10a with calcium and 8

Question 14

Extrinsic Pathway

Answer 14

TF(3) converts VII -> VIIa

VIIa converts 10-> 10a

Question 15

Function of 10a

Answer 15

With 5 and calcium converts prothrombin into thrombin
Thrombin converts fibrinogen into fibrin
Factor 13a helps fibrin form stable clot
13a comes from thrombin producing 13

Question 16

APTT

Answer 16

Intrinsic = 12,11,9 with 8 = 10

Question 17

PT

Answer 17

Extrinsic = TF + 7 = 10

Question 18

Bleeding Time

Answer 18

Platelet Function

3-10 mins

Question 19

Thrombin Time

Answer 19

Common Pathway

Question 20

Severe haemophillia treatment

Answer 20

Factor VIII Prophylactic

Question 21

Mild and moderate Haemophillia treatment

Answer 21

DDAVP for mild haemolphillia A

Factor concentrate

Question 22

What holds plasminogen in its state

Answer 22

Alpha 2 antiplasmin

Question 23

What converts plasminogen -> plasmin?

Answer 23

tissue plasminogen activator

Released by endothelial cells

Question 24

Antithrombin inhibits what?

Answer 24

Thrombin
VII
IX
Xa
XI

Question 25

Examples of a SERPIN

Answer 25

Antithrombin | serpin protease inhibitor

Question 26

Increased anti-thrombin activity

Answer 26

Heparin 5-10,000 fold Stops clot

Question 27

Protein S

Answer 27

Co-factor of protein C

Question 28

Protein C and S

Answer 28

Inactivate Va and VIIIa

Question 29

Increased Protein C activity

Answer 29

Thrombomodulin | 20,000

Question 30

Consanguinity

Answer 30

Increases incidence of rare bleeding disorders 10-20 x

Question 31

Prothrombin Deficiency

Answer 31

XI, VII, V, X, II

Question 32

Factor XI deficiency

Answer 32

Jewish Avoid transexamic acid Tx = factor conc

Question 33

Factor VII deficiency

Answer 33

Low dose recomb fVIIa 15-30ug/kg Levels rise in pregnancy mild don't need replacement

Question 34

Factor V deficiency

Answer 34

Check plasma VIII levels | Tx = FFP plasma solvent detergent = octaplas

Question 35

Factor V and VIII

Answer 35

ERGIC = abnormality of cellular transport | Tx - VIII, DDAVP, plasma

Question 36

Fibrinogen Deficiency

Answer 36

Umbilical cord bleeding Pregnancy abrupt miscarriage, abruption, PPH Tx = fibrinogen replacement, aim >1 during pregnancy, aim >2 for delivery

Question 37

Factor II

Answer 37

Vitamin K dependent factor | Tx = plasma, PTCC

Question 38

Factor X deficiency

Answer 38

Prophylaxis = PTCC, plasma

Question 39

Factor XIII deficiency

Answer 39

- bleeding from birth - prolonged umbilical stump bleeding - Tx = cryo or plasma

Question 40

Vitamin K Dependent Factor Deficiency

Answer 40

II, VII, IX, X PTCC Plasma Check vitamin c as neonate = haemorrhagic disease of newborn