Haemophilia Flashcards
haemophilia A and B - what factor deficient?
A =8
B = 9
inheritance
x linked recessive
males or females?
males
females - affected father and carrier mother
presentation
bleed excessively to minor trauma
gums, GI tract, haematuria
neonate presentation
intracranial haemorrhage
haematomas
cord bleeding
classic bleeding feature for exams
haemarthrosis
bleeding into joints and muscle
diagnosis
bleeding scores
coagulation factor assays
genetic testing
management
IV infusion clotting factors
problem with replacing clotting factors
can form antibodies against factor - treatment become ineffective
treating acute bleeding
infuse factor
desmopressin - release vWF
antifibrinolytics = tranexamic acid
what is prolonged?
APTT
repeated joint bleeding
joint destruction
what is petechial bleeding a sign of?
platelet disorder
what % of clotting factor required to get symptoms?
less than 50%
when are clotting factors given?
mild/moderate - when needed
severe = prophylactically
