DISARTICULATION OF A KNEE-JOINT IN A HEMOPHILIAC WITH HIGH INHIBITOR TITER

Disarticulation of a knee joint in an 8-year-old haemophilia A patient with high inhibitor of 3450 Bethesda units (BU) is described. He had an infected compound fracture of the tibia and fibula. Surgery was suc cessfully performed after extensive plasma exchange; administration of immunosuppressive agents such as cyclophosphamide, methylprednisolone , intravenous immunoglobulin and cyclosporine were combined with a loa ding dose of 100 units kg(-1) of factor VIII concentrate, followed by continuous infusion of 16 units kg(-1) h(-1) of factor VIII in the for m of factor VIII concentrate and cryoprecipitate for 7 days and decrea sed to 8 units kg(-1) h(-1) in the form of cryoprecipitate for 19 more days. During the Ist to 7th post-operative days, the lowest factor VI II inhibitor was 18 BU and the factor VIII level ranged from < 1-2.1 I U dL(-1). On the 9th and 13th post-operative day, although the inhibit or rose to 330 and 2700 BU, respectively, there was no serious bleedin g. The suture was removed on the 21st post-operative day. The inhibito r spontaneously decreased to 550, 232 and 14 BU at 1, 7 and 10 months, respectively.