臨床検査55巻11号

2011年10月発行

特集 ここまでわかった自己免疫疾患 各論 4．そのほかの自己免疫疾患

3) 血栓性血小板減少性紫斑病

著者： 山口雄亮¹ 森木隆典² 村田満³

所属機関： ¹慶應義塾大学大学院医学研究科 ²慶應義塾大学・保健管理センター ³慶應義塾大学・医学部臨床検査医学

ページ範囲：P.1220 - P.1227

血管内皮細胞で産生され血中に放出されたunusually large VWF multimerは，血小板との結合能が高く容易に血管内血栓を形成するが，ADAMTS13で適度に切断されることにより制御されている．TTPはADAMTS13活性が低下することが病態と強く関連しており，先天性TTPではADAMTS13遺伝子変異が認められ，後天性TTPでは多くの症例でADAMTS13に対する自己抗体が検出される．近年，ADAMTS13活性測定法や自己抗体の解析が進展し，TTPの診断，予後，治療効果の判定に大きく貢献している．

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