Immune thrombocytopenia in severe hemophilia A treated with high-dose intravenous immunoglobulin.

Five patients with severe hemophilia A receiving long-term treatment with commercial factor VIII concentrates developed severe immune thrombocytopenia (ITP, platelet counts less than 20 X 10(9)/l). Concomitantly, they presented with a marked elevation of serum IgG concentrations (mean, 2364 mg/dl;range, 1712-2954 mg/dl). In four patients, the T helper to suppressor cell ratio was below 1. Treatment with high-dose intravenous immunoglobulin (IgG, 7s) at doses of 0.2g (n = 2) or 0.4g (n = 3) per kg of body weight on 5 consecutive days was effective immediately. The bleeding tendency ceased and platelet counts rose transiently. In three cases, treatment was repeatedly effective and patients underwent uneventful splenectomies. Thus, high-dose IgG therapy may serve as a life-saving agent in patients with severe hemophilia complicated by ITP.