血栓性血小板減少性紫斑病および血球貪食症候群に対するアフェレシス療法(<特集>腎・血液領域の注目すべき疾患-アフェレシス療法のエビデンスとガイドライン-)

概要

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The condition of thrombotic thrombocytopenic purpura (TTP) is formed by aggreation of platelet thrombi in small vessels of the whole body and leads to minute vessel hemolysis anemia (microangiopathic hemolytic anemia; MAHA), which causes organ damage. Recently, the physiological conditions of TTP based on the severe lack of von Willebrand factor (vWF)-cleaving protease (ADAMTS13) has become clear. Therefore, the utility of apheresis therapy in the treatment of TTP became clear. On the other hand, hemophagocytic syndrome (HPS) is characterized in hemophagocyte with the macrophage in the bone marrow. In the condition of HPS, excessive production of cytokines is a cause. Therefore, the plasma exchange therapy is useful for the removal of the cytokine.
日本アフェレシス学会の論文
2006-02-28