Inherited bleeding disorders Flashcards
1
Q
What factor is deficient in haemophilia A?
A
Factor VIII
2
Q
What factor is deficient in haemophilia B?
A
Factor 9
3
Q
Symptoms for haemophilia?
A
- Asymptomatic / spontaneous bleeding
- Prolonged bleeding - after trauma / surgery
- Haematomas
- Muscle haematomas. haemophilic pseudotumours
- GI bleeding
- Haematuria
- Severe epistaxis
- Painful haemarthrosis - knee most common
- Intracerebral haemorrhage
4
Q
What would be shown on coagulation screen for haemophilia?
A
- Normal platelet count
- Normal prothrombin time
- Extrinsic pathway not affected
- Prolonged partial thromboplastin time
- Intrinsic pathway affected
5
Q
What is von Willebrand disease?
A
Most common inherited bleeding disorder
6
Q
What history and symptoms may indicate von Willebrand disease?
A
- Mucosal membrane bleeding - eg. nosebleeds, heavt periods
- Excessive blood loss from superficial cuts + abrasions
- Haemorrhages after trauma + surgery
7
Q
What could the lab results say about VWD?
A
- Low Factor VIII levels
- APTT prolonged
- Von Willebrand factor levels low
- Platelet count normal
- Except for type 2B disease = low
