阻止因子（インヒビター）を有する血友病Ａ（Ｈｉｇｈ　Ｒｅｓｐｏｎｄｅｒ）に対するＤｏｕｂｌｅ　Ｆｉｌｔｒａｔｉｏｎ　Ｐｌａｓｍａｐｈｅｒｅｓｉｓの試み

概要

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Double Filtration Plasmapheresis (DFPP), one of the new methods of therapeutic plasmapheresis, was applied to a high-responder of haemophilia A with IgG inhibitor, and its effectiveness as well as its side effects were discussed, as no papers concerning the application of DFPP to haemophiliacs with inhibitors could yet be found in the literature.Case: H.I., 19 y.o., male, 47 kg body weight. He suffered from intramuscular bleeding in his left calf. As a high-responder of haemophilia A (severe type) showing maximal serum inhibitor level as high as 255 Bethesda Unit/ml, the "bypass therapy" using prothrombin-complex concentrate applied could not significantly improve his haemostatic status. In order to lower his serum inhibitor level, DFPP was put into practice in two successive days, treating his own plasma 2,700 ml and 1,500 ml respectively, resulting in a significant fall of his serum inhibitor level down to 7.9 from 14.0 B.U./ml without any rebound phenomenon. Thereafter, the "bypass therapy" alone could successfully maintain his improved haemostatic condition. As to the side effects of DFPP, only slight hypotension and haemolysis in the circuit tube were recognized, which needed no particular management.Hereafter, DFPP might become one of the useful means in the management of haemophiliacs with inhibitors.
一般社団法人日本血液学会の論文