REPORT OF 2 CASES OF ACQUIRED VONWILLEBRA ND-DISEASE AND ONE OF ACQUIRED HEMOPHILIA-A

We report three patients with acquired inhibitors against F VIII:C/F v W:Ag complex. Two patients had acquired hemophilia A. The three patien ts presented with bleeding diathesis. Case 1 was a 19 years old woman with Graves-Basedow disease; case 2 was a 40 years old woman with syst emic lupus erythematosus of four years; and case 3 a 38 years old woma n who had had rheumatoid arthritis for five years and was in her 3d mo nth postpartum. The F VIII:C level was below 8 U/dL in all cases. The F vW:Ag, ristocetin cofactor and platelet aggregation with ristocetin were diminished in the two cases with von Willebrand. Inhibitor to F V III:C was 50, 38 and 20 Bethesda units, respectively, for cases 1, 2 a nd 3. The three patients showed clinical response to DDAVP and cryopre cipitates with partial response in laboratory tests. All patients resp onded to corticosteroid treatment, but immnunosuppressive treatment wa s necessary in case 3.