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

Abstract

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 patient s 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. (Journal of Hematopoietic Cell Transplantation Vol. 1 No. 1; 24-28, 2012) BackgroundPure red cell aplasia (PRCA) and autoimmune hemolytic anemia (AIHA) are rare complications that follow allogeneic hematopoietic stem cell transplantation (HSCT).1 3 PRCA has occurred after blood type-mismatched allogeneic HSCT, in which case the remaining isohemagglutinin in the recipient plasma is thought to suppress erythropoiesis. 4 With regard to AIHA, continuous suppression of cellular immunity after allogeneic HSCT is thought to affect humoral immunity, yielding the production of autoantibodies which contribute to hemolysis. 2,5,6 Here we report a case of PRCA followed by Evans syndrome that developed after unrelated donor allogeneic HSCT from a blood type-matched, human leukocyte antigen (HLA) 6/6 matched donor. Case ReportThe patient was a 29 year-old Japanese woman without c a s e s ( 2 . 0 % ) o f p a n c r e a s t r a n s p l a n t a t i o n u s i n g immunosuppression with alemtuzumab, mycophenolate mofetil, and daclizumab. 10 One factor that may be involved in AIHA and Evans syndrome as well as PRCA is an immune abnormality brought about by excessive immunosuppression, but the causative mechanisms of this remain largely unknown.The case study did not use drugs such as ATG for the However, if this treatment does not yield improvement, administration of ATG or steroids, i. e., treatments that strengthen immunosuppression, can also be effective. 4,13,14 Some reports have noted that performing donor lymphocyte infusion and causing the graft-versus-plasma cell effect can conversely be effective in such cases. 4,15 In addition, some have reported the eff...