[Thrombotic thrombocytopenic purpura which was effectively treated by plasma exchange therapy--involvement of vWF and endothelial cell injury].

A 49-year-old man with a one-week history of general fatigue and several other symptoms, including hematuria, numbness of the mouth, anemia and thrombocytopenia, was admitted because of an episode of convulsions and unconsciousness after blood transfusion. A diagnosis of thrombotic thrombocytopenic purpura (TTP) was then made, and treatment with steroids, anti-platelet agents, transfusion of fresh frozen plasma was started. However, since no improvement was seen, on the third day of admission, treatment with plasma exchange was instituted (total plasma exchange volume was 18.1 iota), and his clinical and hematological conditions improved markedly. Since then, he has been in a remission state for about three years. Laboratory examinations during the acute phase showed increase of vWf: Ag, decrease of RCof/vWf: Ag, increase of vWf large multimers and a high endothelial cell injury activity by the patient's serum. In the next day following the plasma exchange therapy, RCof/vWf: Ag improved, but not to the normal range. One and a half years later, while in the remission phase, the vWf multimers and endothelial cell injury activity normalized. Thus, these findings show further evidence on the involvement of endothelial cell injury and vWf in the pathogenesis of TTP.