Many patients with thrombotic thrombocytopenic purpura (TTP) satisfactorily respond to plasma therapy (plasmapheresis and/or plasma infusion). Some, however, respond either not at all or only transiently and incompletely. Evidence indicates that platelets and endothelial cell-derived unusually large von Willebrand factor (ULvWF) multimers, as well as the largest vWF multimers in plasma, form thrombi which are deposited in the microvascular circulation. Accordingly, platelet transfusions are avoided unless there is intra-cranial or other life-threatening hemorrhage. The largest plasma multimers of vWF are, however, replenished by the infusion of large volumes of whole plasma. We postulated that under conditions of massive plasma replacement, plasma depleted of the largest multimers of vWF might be preferable for the treatment of TTP episodes. The largest vWF multimers sediment in the cryoprecipitate, and the cryoprecipitate-poor fraction of plasma (cryosupernatant) is depleted of these forms. Seven patients responding inadequately to intensive plasma therapy were switched to receive cryosupernatant in place of whole plasma. All patients improved quickly following this change in therapy, and the TTP syndrome resolved in all seven.
- Von Willebrand factor
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