Intravenous immunoglobulin therapy for acquired coagulation inhibitors: a critical review

Intravenous immunoglobulin (IVIG) therapy has been used for autoimmune diseases and disorders involving autoantibodies, including coagulation inhibitors. In this review, we have evaluated the efficacy and safety of IVIG therapy for acquired coagulation inhibitors, including factor VIII inhibitor, and for acquired von Willebrand syndrome on the basis of 44 reports published between 1965 and 2005. Among 35 patients with factor VIII inhibitor, we estimated the efficacy of IVIG therapy alone (which includes complete remissions and partial responses with a clinical benefit) to be 30% (11 cases), whereas the response to combination therapy with IVIG plus immunosuppressive agents (eg, corticosteroid, cyclophosphamide) seemed to be better (approximately 70%, 33/45 cases) than with IVIG therapy alone. In acquired von Willebrand syndrome, the efficacy of IVIG therapy was estimated to be 30%. The response to IVIG therapy appears to occur rapidly, and coagulation inhibitors seem to be neutralized immediately. Moreover, severe complications or side effects rarely occur during IVIG treatment. IVIG therapy thus may be considered one choice for treating acquired coagulation inhibitors, although its efficacy improves when used in combination with immunosuppressive agents.