Hemophilia history

Inherited deficiencies of the clotting system that result in bleeding are found in humans. The most common is deficiency of factor VIII, causing hemophilia A, an X chromosome-hnked disease that has played a major role in the history of the royal families of Europe. Hemophilia B is due to a deficiency of factor IX its clinical Feamres are almost identical to those of hemophilia A, but the conditions can be separated on the basis of specific assays that distinguish between the two factors. 

Two patients (aged 60 and 73 years) with mild and moderate hemophilia respectively, without a family history of inhibitor formation, were exposed to factor VIII for more than 50 days. An inhibitor developed in both patients after an intermediate factor VIII product was replaced by a high-purity factor VIII product, which was given by continuous infusion during a surgical procedure. 

The diagnosis of hemophilia should be considered in any male with unusual bleeding. A family history of bleeding is also helpful in the diagnosis, but this is absent in up to one-third of patients. Brothers of patients with hemophilia should be screened sisters should have carrier testing. 

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