抄録
A typical case of thrombotic thrombocytopenic purpura (TTP) was reported in which an immunological mechanism was suspected to be relevant to its etiology.
A 31-year-old man had a sudden onset of general malaise, vertigo, headache and bleeding tendency. He was immediately admitted to our hospital because of convulsion and disturbance of consciourness. Laboratory examinations revealed obvious anemia and hemorrhagic diathesis in association with microangiopathic hemolytic anemia characterized by red cell fragmentation and nucleated red blood cells. Coagulation time, fibrinogen, factor VIII and antithrombin III were within normal limits while thrombocytopenia and a mild elevation of FDP were observed. He was treated with prednisolone, fresh frozen plasma and heparin on a diagnosis of TTP, but bleeding tendency and coma progressively increased, and he died 11 days after the onset of symptoms.
Aspiration autopsy specimens of the spleen revealed multiple thrombi at the small arteries and the proliferation of endothelial cells of these arteries. In addition, a deposition of IgM and a small amount of C3 was observed in the endothelium of small splenic arteries.
