title: Clinical features and risk factors of pure red cell aplasia following major ABO-incompatible allogeneic hematopoietic stem cell transplantation.
abstract: The objective of this paper was to study the incidence, risk factors, clinical outcome, management and prevention of pure red cell aplasia (PRCA) following major ABO-incompatible allogeneic hematopoietic stem cell transplantation (allo-HSCT). We retrospectively analyzed 11 cases of PRCA from a series of 42 patients undergoing major ABO-incompatible allo-HSCT from April 1997 to December 2005. Eleven out of the 42 patients developed PRCA (26.1%). All the 11 cases of PRCA were in blood group O recipients of grafts from blood group A donor (n = 9) or blood group B donor (n = 2). The following factors were associated with an increased risk of PRCA: (1) blood group O recipient; (2) blood group A donor; and (3) blood group O/A in recipient/donor pair. Only blood group O/A in recipient/donor pair was identified as being significantly associated with the occurrence of PRCA by multivariate analysis. Six patients who received donor-type plasma exchange did not develop PRCA and among them 5 cases were the blood group O recipients. Eight patients obtained spontaneous remission and in the remaining 3 patients 2 with long-lasting PRCA were successfully treated with plasma exchange with donor-type plasma replacement and the other one who was also complicated by EBV-associated lymphoproliferative disorder (EBV-PTLD) responded rapidly to anti-CD20 monoclonal antibody and achieved complete resolution of clinical finding and symptom of both EBV-PTLD and PRCA. We conclude that blood group A/O in donor/recipient pair is identified as being significantly associated with the occurrence of PRCA by multivariate analysis. Donor-type plasma exchange and anti-CD20 monoclonal antibody is an effective approach for the treatment of PRCA. PRCA could be prevented by plasma exchange prior to transplantation.
authors: Department of Hematology, First Affiliated Hospital of Jinan University, Guangzhou, People's Republic of China.
authors: Kang-er
authors: KE
authors: Zhu
authors: Jun-ping
authors: JP
authors: Li
authors: Tao
authors: T
authors: Zhang
authors: Juan
authors: J
authors: Zhong
authors: Jie
authors: J
authors: Chen
mesh_terms: D000017:ABO Blood-Group System / Q000235:genetics / Q000276:immunology; D000328:Adult; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D058846:Antibodies, Monoclonal, Murine-Derived; D018951:Antigens, CD20 / Q000276:immunology; D001787:Blood Group Incompatibility / Q000209:etiology*; D002066:Busulfan / Q000494:pharmacology; D002675:Child, Preschool; D016572:Cyclosporine / Q000627:therapeutic use; D020031:Epstein-Barr Virus Infections / Q000150:complications; D005260:Female; D006085:Graft Survival; D006086:Graft vs Host Disease / Q000517:prevention & control; D018380:Hematopoietic Stem Cell Transplantation / Q000009:adverse effects*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007938:Leukemia / Q000601:surgery; D008232:Lymphoproliferative Disorders / Q000209:etiology / Q000628:therapy; D008297:Male; D008727:Methotrexate / Q000627:therapeutic use; D008875:Middle Aged; D009173:Mycophenolic Acid / Q000031:analogs & derivatives / Q000627:therapeutic use; D010951:Plasma Exchange; D012010:Red-Cell Aplasia, Pure / Q000453:epidemiology / Q000209:etiology* / Q000628:therapy; D012075:Remission, Spontaneous; D012189:Retrospective Studies; D012307:Risk Factors; D000069283:Rituximab; D019172:Transplantation Conditioning; D014184:Transplantation, Homologous / Q000009:adverse effects*; D014740:Vidarabine / Q000031:analogs & derivatives / Q000494:pharmacology; D014916:Whole-Body Irradiation; D017086:beta-Thalassemia / Q000601:surgery
chemical_list: D000017:ABO Blood-Group System; D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D018951:Antigens, CD20; D007166:Immunosuppressive Agents; D000069283:Rituximab; D016572:Cyclosporine; D014740:Vidarabine; D002066:Busulfan; D009173:Mycophenolic Acid; C024352:fludarabine; D008727:Methotrexate