Introduction to Bleeding Disorders/Heritable Bleeding Disorders

Question 1

How does haemostatic plug formation occur?

Answer 1

1) Aggregation - platelets come together to form a clot that forms a temporary seal over the break
2) Coagulation - platelet plug is reinforced with fibrin

Question 2

When does haemostatic plug formation form?

Answer 2

when there is injury to the blood vessel wall

platlets adhere to damaged endothelium = activation

Question 3

When does the intrinsic method of coagulation occur?

Answer 3

when blood is in contact with a foreign surface

Question 4

When does the extrinsic method of coagulation occur?

Answer 4

Tissue factor exposed when injury occurs

Question 5

Draw out the coagulation cascade

Answer 5

[see slide]

Question 6

What is the role of plasmin?

Answer 6

breaks down fibrin in clots

mopped up as can break down other factors

Question 7

What are the signs of vascular/platelet defect?

Answer 7

Petechiae and superficial bruises
Skin and mucose membranes
Spontaneous bleeds
Bleeding immediate, prolonged and non-recurrent

Question 8

What are the signs of coagulation defect?

Answer 8

Deep spreading haematoma
Haemarthrosis - bleeding into joint spaces
Retroperitoneal bleeding
Bleeding prolonged and often recurrent

Question 9

What is petechiae?

Answer 9

Red or purple spot on the skin caused by a bleed from broken capillary blood vessels
Does not blanch with pressure
not palpable

Question 10

What is a common symptom of bleeding disorders?

Answer 10

Epistaxis (nose bleed)

Question 11

What is Von Willebrand disease?

Answer 11

a deficiency or abnormal structure of the Von Willebrand factor
This means that their blood cannot clot properly
prolonged or excessive bleeding

Question 12

Is type I, II or III of Von Willebrand disease the most severe?

Answer 12

Type III - very low levels or no Von Willebrand factor

Question 13

Which type of Von Willebrand disease occurs when the factor doesn’t work properly?

Answer 13

Type II

Question 14

Which types of Von Willebrand disease are dominant?

Answer 14

Type I and II

Question 15

Why can there be an overlap of vessel wall/platelet deficiency symptoms and coagulation symptoms in Type III Von Willebrand disease?

Answer 15

Von Willebrand factor carries Factor 8 in the blood
therefore a deficiency of Von Willebrand factor means a deficiency of factor 8 in the blood = coagulation deficiency symptoms

Question 16

What is the most common heritable bleeding disorder?

Answer 16

Von Willebrand disease

Question 17

What blood group generally has lower levels of Von Willebrand factor?

Answer 17

O

Question 18

How is haemophilias inherited?

Answer 18

X linked recessive

typically expressed in males and carried in females

Question 19

What factor is deficient in Haemophilia A?

Answer 19

Factor 8

Question 20

What factor is deficient in Haemophilia B?

Answer 20

Factor 9

Question 21

What types of bleeds can occur in haemophilia?

Answer 21

Spontaneous/Post traumatic
Joint Bleeding = Haemarthrosis
Muscle Haemorrhage 
Soft Tissue
Life Threatening Bleeding

Question 22

What is Hemophilic Arthropathy?

Answer 22

permanent joint disease occurring in haemophilia sufferers as a long-term consequence of repeated haemarthrosis

Question 23

What is inhibitor development?

Answer 23

the body developies an antibody to the product used to treat or prevent bleeding episodes
more common in haemophilia A