Human ABO blood type, which was discovered in 1901 by Tagareli and Landsteiner , is a major immunological barrier in organ transplantation because of a development of severe rejection due to the humoral reaction between A or B antigens and anti-A or anti-B antibodies. ABO-incompatible kidney transplantation was first performed in 1952 by Hume et al. for the patient of renal failure . The transplanted kidney, however, did not function at all. Starzl et al. also performed ABO-incompatible kidney transplantation in four patients in 1964 and succeeded long-term graft survival in one patient [3, 4]. However, since a success rate was extremely low, ABO-incompatible kidney transplantation generally ceased to be performed . Thereafter, in 1981, Slapak et al. reported the efficacy of plasmapheresis for the rejection after kidney transplantation with ABO incompatibility . Alexandre et al. first performed ABO-incompatible kidney transplantation using the designed protocol using plasma exchange for pretransplant removal of anti-A and anti-B antibodies and splenectomy for long-term graft survival [7-9]. In Japan, Takahashi et al. have introduced the first ABO-incompatible kidney transplantation in 1989 using double-filtered plasmapheresis (DFPP) with immunoabsorption for the removal of antibodies and splenectomy which resulted in long-term graft survival [10-14]. This clinical success encouraged us to promote ABO-incompatible living kidney transplantation in Japan, and 2,218 patients underwent ABO-incompatible living kidney transplantation from January 1 to December 31 . The number of ABO-incompatible living kidney transplantation has increased year and year, and currently, about 25 % of living kidney transplantation is ABO-incompatible transplantation in Japan. In addition to kidney transplantation, ABO-incompatible transplantation has been introduced into liver transplantation  and pancreas transplantation  in Japan. ABO-incompatible donor is considered as an immunologically marginal donor for the recipient. Pretreatment for the recipient, which includes pretransplant immunosuppression and improved desensitization protocol as well as careful monitoring of the antibody titer both before and after transplantation, achieved the improved graft survival that is almost equal to that in ABO-compatible kidney transplantation. Especially in Japan, ABO-incompatible kidney transplantation has become widely adopted due to a severe shortage of deceased donors. In this chapter, we describe the current status of ABO-incompatible kidney transplantation in Japan and our experiences of simultaneous pancreas and kidney transplantation from ABO-incompatible live donors in a single institution.
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