TTP/HUSに対するアフェレシス(<特集>腎疾患とアフェレシス-最近の知見-)

概要

論文の詳細を見る
Thrombotic microangiopathy (TMA) comprises thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS). After the discovery of von Willebrand factor cleaving protease (vWF-CP or ADAMTS13), the definitions of TMA have greatly changed; one with decrease in ADAMTS13 activity is TTP, one without a decrease in its activity is HUS. Simple plasma exchange (PE) with fresh frozen plasma (FFP) supplementation is the mainstay of the treatment of TMA especially idiopathic TTP. The efficacy of PE is thought to be derived from the supplementation of ADAMTS13 and removal of its inhibitor-usually an auto-antibody against ADAMTS13. After introducing PE into the treatment of TTP, the prognosis has been improved sharply. Moreover, hemodialysis tandemly connected to PE might decrease the adverse effects of PE such as hypocalcemia. However, there remain several types of TMA with certain etiology for which the application of PE is not considered; these include TTP caused by cancer, organ transplantation, or anti-cancer drugs, as well as HUS that follows enterohemorrhagic E. coli infection. Although some limitations exist such as relapsing or refractory types of TTP as well as availability of PE, starting PE should be considered for the patient who suffers from hemolytic anemia and thrombocytopenia of unknown origin.
2011-05-31