TTP/HUSの病態とアフェレシス治療の意義(<特集>21世紀のアフェレシス-作用機序と臨床効果のエビデンス)

概要

論文の詳細を見る
Recent advances in the research of the pathogenesis of thrombotic thrombocytopenic purpura(TTP)have clarified that the von Willebrand factor(vWF)cleaving metalloprotease plays a central role in the development of the disease. The decreased protease activity because of an inherited deficiency or an. acquired inhibitor fails to cleave of unusually large vWF(UIvWF). These ULvWF multimers cause platelet aggregation, leading to multisystem organ damage and failure. The removal of ULvWF multimers and the inhibitor by therapeutic plasma exchange (TPE)and the addition of vWF cleaving protease by TPE and plasma infusion are the most suitable plasma therapies for each disease type.
日本アフェレシス学会の論文
2001-02-28