Comprehensive approach to hemophilia

Abstract

Hemophilia A, B are X-linked recessive bleeding disorder that typically results from a deficiency of clotting factor VIII (FVIII) and factor IX (FIX). The severity of the disease is determined according to the FVIII and FIX levels. Hemophilia A and B have similar symptoms and are both characterized by bleeding, particularly in large joints such as ankles, knees, elbows. Recurrent bleeding in joints eventually causes progressive hemophilic arthropathy. Life-threatening hemorrhages may occur rarely. Treatment of hemophilia has improved significantly in recent years with clotting factor concentrates. The average life expectancy was <40 years until the 1960s, but with better accessing clotting factor concentrates and prophylactic replacement of the missing factor, today hemophilia patients can perform social activities like other healthy individuals and live with an almost normal life expectancy. The future of hemophilia seems bright with gene therapy and new non-replacement treatments.

Keywords

• bleeding
• factor concentrates
• factor VIII
• factor IX
• hemophilia

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