PREDNISONE AND LOW-DOSE ACTIVATED PROTHROMBIN COMPLEX CONCENTRATES FOR FVIII INHIBITOR IN NONHAEMOPHILIC PATIENTS

Eight nonhaemophilic patients with factor VIII (FVIII) inhibitors were reported. There was no difference in sex distribution. Median age at diagnosis was 62 years (ranging from 14 to 73 years). No associated di sorders were revealed and all the patients were presented with severe muscular or arthral bleeding. Inhibitor titre was measured by the Beth esda method, which were 6.4, 126.0, 155.0, 4.8, 56.0, 13.5, 35.0 and 1 50.0 BU mL(-1), respectively, at diagnosis. FVIII:C levels were less t han 1 U dL(-1) in seven patients and less than 2 U dL(-1) in one patie nt. The median vWF:Ag level was 210% (ranging from 80% to 340%). All t he patients had good response to activated prothrombin complex concent rates for acute bleeding episodes and prednisone for inhibitor elimina tion. Inhibitors completely eliminated in seven patients within a foll ow-up duration over 1 year, and one patient died of intracranial haemo rrhage when her inhibitor titre decreased to 4.5 BU mL(-1) and FVIII:C increased to 21 U dL(-1).