CP37 - Heritable Bleeding Disorders

Question 1

when is tissue factor expressed

Answer 1

TF is expressed when damages to cells are done and will be presented on the surface of blood vessels

Question 2

what activate factor 7 in blood ?

Answer 2

tissue factor

Question 3

when does common pathway start

Answer 3

when factor 1 0is activated

Question 4

when will intrinsic pathway of coagulation pathway start?

Answer 4

when blood come into contact with foreign surfaces

Question 5

which factors is activated first when blood come into contact with foreign objects

Answer 5

factor 12

Question 6

what does coagulation screen tests consist of ?

Answer 6

prothrombin time, activated partial thromboplastin time, thrombin clotting time or euglobulin lysis time

Question 7

how does the prothrombin time test work?

Answer 7

time taken for clot to appear after TF and Ca+ are added to the sample - looking at the extrinsic pathway

Question 8

how does the activated partial thromboplastin time work?

Answer 8

time taken for fibrin clot to appear after the calcium-phospholipid mixture is added. - looking at the intrinsic pathway

Question 9

what are some of the agents present for anti-coagulant?

Answer 9

Protein C, protein S, anti-thrombin III, fibrinolytic system

Question 10

which enzyme breaks down fibrin

Answer 10

plasmin

Question 11

where are the common places for haemorrhage to occur for patient who has platelet/bleeding disorder

Answer 11

mucosal and skin

Question 12

where are the common places for haemorrhage to occur for patient who has coagulation defect

Answer 12

deep muscle & joint bleeds, bleeding more following trauma

Question 13

what is an example for abnormal platelet fucntion

Answer 13

aspirin

Question 14

what is an example for abnormal vessels wall

Answer 14

scurvy - Vit C important for production of collagen which is an important component of cell wall

Question 15

what is an example for abnormal interaction between platelets & vessel wall

Answer 15

von Willebrand disease - von Willebrand factors - act like glue

Question 16

which type of von Willerbrand disease is the most common

Answer 16

type 1

Question 17

what is the problem in type 1 von Willerbrand disease

Answer 17

low production of normal VWF.

Question 18

what is the problem in type 2 von Willerbrand disease

Answer 18

normal amount of VWF but abnormal quality

Question 19

what is the problem in type 3 von Willerbrand disease

Answer 19

almost absent of VWF

Question 20

which coagulating factor does VWF carry?

Answer 20

factor 8

Question 21

what can reduce level of VWF cause

Answer 21

factor 8 deficiency

Question 22

what is the mechanism of inheritance for von Willerbrand disease?

Answer 22

autosomal dominant inheritance

Question 23

what is the pattern of bleeding for von Willerbrand diseaese?

Answer 23

mucocutanous bleeding including menorrhagia

Question 24

which blood group have a lower VWF?

Answer 24

group O

Question 25

what are the treatment of von willebrand disease

Answer 25

tranexamic acid - antifibrinolytics

DDAVP (desompressin) - release VWF into plasma temporality for type 1 vWD

Question 26

which coagulation factor is deficient in haemophilia A

Answer 26

Factor 8

Question 27

which coagulation factor is deficient in haemophilia B

Answer 27

factor 9

Question 28

what is the mode of inheritance for haemophilia

Answer 28

sex linked recessive

Question 29

which test is prolonged for patients with haemophilia A & B

Answer 29

APTT - affect intrinsic pathway

Question 30

can haemophilia be sporadic?

Answer 30

yes - about 30%

Question 31

what is the pattern of bleeding for haemophilia ?

Answer 31

Spontaneous/Post traumatic
Joint Bleeding = Haemarthrosis
Muscle Haemorrhage 
Soft Tissue
Life Threatening Bleeding – tissue surrounding the airway

Question 32

what are the treatment for haemophilia

Answer 32

replacement of clotting protein - on demand/prophylaxis

DDAVP

antifibrinolytic agents

vaccination vs Hep A and B

Question 33

what are some complication for treatment of haemophilia

Answer 33

transfusion transmitted infection, inhibitor development - enzyme against factor 8 or 9