Pure red cell aplasia and Evans syndrome following hematopoietic stem cell transplantation from blood type-matched unrelated donor

概要

論文の詳細を見る
Pure red cell aplasia (PRCA) and Evans syndrome following allogeneic hematopoietic stem cell transplantation (HSCT) from a blood type-matched donor are very rare. A 29-year-old Japanese woman with hematologic remission of Philadelphia chromosomepositive acute lymphoblastic leukemia underwent bone marrow transplantation from a blood type-matched, HLA 6/6 — matched, unrelated donor in May 2008. Her clinical course after transplantation was favorable, and allowed for a gradual reduction in tacrolimus dosage. However, 12 months after transplantation, she developed PRCA related to allogeneic HSCT. The patientʼs PRCA was alleviated by stopping the dosage reduction for the immunosuppressive therapy and by continued administration of lowdose tacrolimus. Three months after the PRCA diagnosis, the patient developed Evans syndrome, so prednisolone (PSL) treatment was initiated. PSL treatment effectively treated the Evans syndrome, as was evident by the absence of cythemolysis two months later, and no relapse has occurred even after the PSL dose was reduced and then terminated. The fact that PRCA onset in this case occurred after blood type-matched allogeneic HSCT, during the late grafting phase, and in combination with Evans syndrome, suggests that the pathogenic mechanism may differ from that of previously reported cases of PRCA following blood type-incompatible allogeneic HSCT.