血しょう交換により救命し得た血せん性血小板減少性紫はん病（ＴＴＰ）の１症例 本邦報告例の臨床病理学的検討

Abstract

An interesting case of TTP, which responded dramatically well to plasma exchange (P. E.), is presented in detail. Besides, case reports that have appeared in the Japanese literatures in the last decade is summarized and reviewed in terms both of clinical features and of treatment, with emphasis on P. E. as a pertinent therapeutic procedure of the disease.A 46-year-old man with macrohematuria and purpuric skin lesions at his shoulders with a week duration was admitted to the hospital on December 22, 1983, in suspicion of thrombocytopenia and hemolytic anemia presumed to be induced by some medicine prescribed for cold-like symptoms. By the hospital day 3, his consciousness became confused, then the clinical diagnosis of TTP was made. On the hospital day 7, he fell into unconsciousness despite combined use of corticosteroids and antiplatelet agents. Then he was started on P. E. And, on the hospital day 31, when the 13th procedure of P. E. was finished, his consciousness got clear and hematological findings improved. About 60 days after the current admission, almost all indices of hematological examination were within normal limit.In Japan, sixty-three cases of TTP including this case have been reported during the last 10 years. On grounds of clinicopathological studies on these sixty-three cases of TTP in Japan, the following results were obtained: 1) The age of patients with TTP ranged from 8 to 63, with mean value of 33.1.2) Ratio of male to female was 20 to 43.3) Major symptoms which patients had consisted of purpuric skin lesions, hematuria, hemolytic anemia, psychiatric symptoms, febrile episodes, and renal dysfunction. 4) Twenty-five out of sixty-three patients (39.7%) died within a year after the onset of the disease, while thirty-eight (60.3%) survived: Eight among those sixty-three cases received P. E. Complete remission were attained in four out of those eight cases that were given P. E., while two out of those eight cases reached to partial remission. The rest two gained no beneficial effects.Meanwhile, neither corticosteroid, antiplatelet agents nor any combination of these measures could exert any definite beneficial effects. Taken altogether, there have been few case reports of TTP in Japanese literatures so far and a large majority of those cases reported could not survive despite any combined usage of corticosteroid, antiplatelet agents or anticoaglants, whereas, P. E. turned out to be of significant value by comparison with any other regiments of treatment ever used.