[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
low platelets, low fibrinogen. dx?
DIC
raised INR, low platelets and deranged LFTs. dx?
liver cirrhosis
true or false: Von Willebrand’s disease has increased bleeding time, haemophilia does not
true
