Bleeding disorders

Question 1

Half life of Factor VIII?

Answer 1

First dose: 6-8hrs

Subsequent doses: 12hrs

Question 2

Half life of Factor IX?

Answer 2

First dose: 4-6hrs

Subsequent doses: 24 hours

Question 3

As a broad rule, platelet deficiency leads to what type of bleeding?

Answer 3

petechiae

Question 4

As a broad rule, factor deficiencies lead to what type of bleeding

Answer 4

ecchymoses

Question 5

What is the most common form of coagulopathy?

Answer 5

von Willebrand’s disease

estimated frequency 1:100 - 1:1000

Question 6

Functions of von Willebrand factor?

Answer 6

Binds platelets to endothelial structures
Carrier protein for FVIII
Protects FVIIIc from degradation

Question 7

How is von Willebrand disease inherited?

Answer 7

AD

Question 8

Describe the 3 types of von Willebrand disease?

Answer 8

Type 1 (60-80%): protein reduced but not absent
Type 2: abnormal protein
Type 3: absent protein

Question 9

Haemophilia A (or “classic disease”) is caused by which factor deficiency?

Answer 9

FVIII

Question 10

Haemophilia B (or Christmas disease) is caused by which factor deficiency?

Answer 10

FIX

Question 11

Indicators suggestive of Haemophilia during the neonatal period?

Answer 11

Bleeding with cord separation
Bleeding post circumcision
(~2% IVH)

Question 12

Inheritance of Haemophilia?

Answer 12

X-linked recessive

~1/3 spontaneous mutations

Question 13

Based on factor deficiency, what constitutes severe, moderate and mild Haemophilia?

Answer 13

Severe: <1%
Moderate: 1-5%
Mild disease: 5-25%

Question 14

What is Haemophilia C disease and how is it inherited?

Answer 14

Factor XI deficiency

AR inheritance

Question 15

SE of desferroxamine?

Answer 15

ototoxicity