Hemophilia: The Royal Disease. Natalia A Palacio April 2006. Definition. Hemophilia- “love of bleeding” 2 types: A and B Hemophilia A: X linked recessive hereditary disorder that is due to defective or deficient factor VIII. History.
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Natalia A Palacio
A pseudotumor is deforming the cortex of the femur (arrow).
Other ossified masses in the soft tissues (arrowheads) are probably soft-tissue pseudotumors.
PUD is 5 times more common in hemophiliac patients than regular males. Associated with ingestions of NSAIDs for hemarthrosis. Frequent cause of UGIB
Epistaxis, gum bleeding.
Frequently severe hemophiliac can experience hematuria and a structural lesion should be ruled out.
Replacement of F VIII is the cardinal step to prevent or reverse acute bleeding episodes
Sources of F VIII
Bethesda assay: which consists of serial dilutions of plasma is pooled with normal plasma and incubated for 2 hs, then the activity level is measured by coagulation assays. The higher inhibitor titer, the greater the dilution required to demonstrate residual FVIII activity. It is expressed on Bethesda Units: High responders: >5 Bethesda units, low responders <5.
Which Hemophilia was it
A or B????