[Haem] Haemophilia Flashcards
what is haemophilia?
X-linked recessive bleeding disorder which comes in 2 forms: Haemophilia A and Haemophilia B
what is haemophilia A?
factor VIII deficiency
mild - 5% factor VIII
moderate - 1-5% factor VIII
severe - <1% factor VIII
what is haemophilia B?
factor IX deficiency
how does haemophilia present?
- FHx in males of bleeding
- most common: haemarthrosis
what is the 1st line ix for haemophilia?
factor VIII and IX assay
how is a dx of haemophilia made?
clotting
- PT: normal
- APPT: increased
- bleeding time: normal
how are haemophilia pts managed pre-operatively?
- haematology advice
- factor VIII concentrate 30-60 minutes peri-operatively
pt with haemophilia A presents with a severe, life-threatening bleeding from an intracranial haemorrhage. how do you treat the pt?
factor VIII concentrate - aiming levels 100%
*consult haematology for dose
pt with haemophilia A presents with haemarthrosis. how do you treat the pt?
factor VIII concentrate - aiming levels 50%
*consult haematology for dose
what are used as prophylaxis for haemophilia A?
- factor VIII concentrate
- emicizumab
INR - normal APPT - increased PT - normal platelets - normal bleeding time - normal
what is the disorder?
haemophilia
X-linked recessive
INR - normal APPT - increased PT - normal platelets - normal (occasionally low) bleeding time - increased
what is the disorder?
von Willebrand’s disease
autosomal dominant
INR - increased APPT - increased PT - increased platelets - low bleeding time - increased
what is the disorder?
DIC
INR - increased APPT - increased PT - normal/increased platelets - low (with splenomegaly) bleeding time - increased
what is the disorder?
liver cirrhosis
menorrhagia, prolonged bleeding time. dx?
von Willebrand’s disease
