haemophilia Flashcards
what is haemophilia?
a rare disorder in which blood does not clot normally because it lacks sufficient blood clotting proteins ( clotting factors)
CBC is usually normal
causes:
1) genetic
2) acquired spontaneous mutation ( pregnant, cancer, autoimmune, allergic reaction to medications)
what is the difference between haemophilia A, B, C.
a: VIII
B: IX
C: XI
A and B- are X linked recessive
C can be passed down to both females and males.
what is PTT for
to measure VIII, IX, XI and XIII 7 9 11 13
what is PT for
to measure I, II, V, VII, X
1 2 4 6 10
what is fibrinogen for
I
management of hemophilia
DDAVP ( desmopressin ) to stimulate factor VIII 7 release
self infusion at home,
replacement of clotting factor VIII IX from donated human blood or recombinant clotting factors.
complications of hemophilia
deep internal bleeding
severe anemia
infection from frequent infusion
adverse reaction from clotting treatment