Late response to cyclosporine in refractory thrombotic thrombocytopenic purpura.

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Abstract
Plasma exchange (PEX) with fresh frozen plasma, usually in association with steroid therapy, has been shown to be the firstline treatment of thrombotic thrombocytopenic purpura. It works by removing ultralarge von Willebrand factor (vWF) multimers and inhibitory antibody and by supplying normal protease. For patients with disease refractory to PEX, there is no standardized treatment. Limited and sporadic success with different therapies (vincristine sulfate, prostacyclin, intravenous immunoglobulins, splenectomy) has been described. We report the case of a woman who developed refractory disease after an initial response to PEX and despite a very high number of PEX procedures performed. Low activity (Int J Hematol. 2002;76:284-286.