血栓性血小板減少性紫斑病に対する血漿交換療法(<特集>血小板減少症とアフェレシス)

概要

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It is well known fact that plasma exchange (PEx) applying fresh frozen plasma dramatically improved the prognosis of the patients suffering from thrombotic thrombocytopenic purpura (TTP). Before induction of PEx to the treatment of TTP, mortality rates were in excess of 90% but plasma exchange applying FFP as a replacement fluid reduced the mortality rate to about 20%. Deficiency of ADAMTS13 (a cleaving protease of von Willebrand factor) was demonstrated to be a main pathogenesis of TTP in the 1990s. Removal of autoantibodies of ADAMTS13 and infusion of ADAMTS13 are the main purposes of PEx. Replacement of 1 to 1.5 plasma volume of FFP during a single procedure of PEx is appropriate. Daily PEx is recommended to continue until a few days after recovery of platelet count. Exacerbation during PEx and relapse and resistance occur at a rate of 20 to 50%. It is very important to recognize PEx is a treatment infusing corresponding antigens to the blood containing pathogenic antibodies producing B-Lymphocytes. Improvement of immunosupressive therapy associated with PEx is necessary to achieve better outcome. Rituximab, a chimeric monoclonal antibody against CD20, has been reported to be a good candidate for a breakthrough for this dilemma.
2012-02-29