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Two patients with excessive bleeding associated with “spontaneous” Factor VIII inhibitors were studied. The first, a White male aged 52 years, was treated with high dosage cyclophosphamide and steroids together with plasmapheresis. An antigenic stimulus via concentrate and fresh plasma was given together with the stat dose of cyclophosphamide (1.6gm) together with prednisolone 60mg/day. Repeat plasmopheresis was carried out on two subsequent occasions shortly thereafter. The inhibitor level dropped progressively from 6.6 u/ml to almost unrecordable levels. However, escape from control was associated with the onset of hepatitis. Further therapy with an identical form of treatment failed to subsequently modify the inhibitor level which rose progressively to very high levels. In the second case, a White female aged 79 years, plasmapheresis was not carried out (inhibitor level 2.0 u/ml). Cyclophosphamide and prednisone were given in doses of 20 mg/Kg and 60 mg/day respectively. A Factor VIII antigenic load was given 24 hours before the cyclosphosphamide. Two subsequent cyclophosphamide pulses of similar dosage were given at approximately 10 day intervals without an antigenic stimulus. The patient was then maintained on a small daily dose of cyclophosphamide (50 mg/day). The inhibitor level responded to this therapy resulting in disappearance of the inhibitor and a progressive rise in the Factor VIII to levels greater than normal (170%). Our experience with these two cases suggests that a more rational approach to immunotherapy in the second case resulted in a sustained satisfactory immunosuppressive response.

The Management of two Cases of “Spontaneous” Factor VIII Inhibitors with high Dose Cyclophosphamide