HEMOPHILIA
Dr Ira Shah
Medical Science Department, Pediatric Oncall, Mumbai
Address for Correspondence: Dr Ira Shah, 1, B Saguna, 271, B St Francis Road, Vile Parle {W}, Mumbai 400056
Clinical Problem :
A 10 years old boy who is a diagnosed case of hemophilia {Factor VIII levels Less than 1 percent} not on regular Factor VIII therapy presented with abdominal pain and black stools since 1 day. There is no fever or bleeding from any other site. On examination, he was pale, had tachycardia {Heart rate = 130, min} with bilateral basal crepitations and hypotension {blood pressure = 80, 60 mm of Hg}. He had tender hepatomegaly and no bruises or deep tissue bleeding. Other examination findings were normal. Investigations showed hemoglobin of 3.7 gm, dl, WBC count of 22,700, cumm {70 percent polymorphs, 30 percent lymphocytes} and normal platelet count. Partial thromboplastin time {PTT} was 87 seconds and prothrombin time {PT} was normal. He was treated with IV Fluids and dobutamine. Factor VIII concentrate could not be given due to unaffordability.
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To stop bleeding, what can be given – cryoprecipitate or fresh frozen plasma__?
Discussion :
This child was severe hemophilia and for abdominal bleedings leading to congestive cardiac failure, ideal replacement should be recombinant Factor VIII concentrates {50 IU, kg}. However if recombinant Factor VIII is not available, alternative in form of blood component therapy should be used. Cryoprecipitate is the preferred blood component as it is rich in Factor VIII whereas Fresh Frozen Plasma {FFP} is not useful as it contains decreased Factor VIII. Thus giving FFP is not going to make much of a difference.
E-published: April 2013 Art No. 22
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