April 20, 2009

A 6 years old boy with history of easily bruising present to your hospital with bilateral knee joint swelling.


Bleeding time: 4 min (3-9 min)
Platelet count: 300 X 109/L (150-450 X 109/L)
PT : 14 s (12-16 s)
APTT : 48 s (23-31s)



1) Abnormality in which chromosome usually associated with this condition (2m)
2) What is the most likely diagnosis of the bilateral knee joint swelling (2m)
3) What type of transfusion will you consider in this patient (2m)
4) Outline the pathophysiology of the current symptoms (4m)



Answer


1) Chromosome X



2) Haemarthroses due to Hemophilia A



3) Replace only needed factor via IV infusion which is factor VIII. To do that, we might consider
A. Clotting factor concentrate
B. Recombinant Factor VIII
C. Fresh Frozen Plasma (FFP)
D. Cryoprecipitate


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