Abstract
Pure red cell aplasia (PRCA) after ABO-incompatible allogeneic hematopoietic stem cell transplantation (HSCT) is caused by persisting host-derived isohemagglutinins directed against donor red blood cell (RBC) antigens. ABO antigen-specific immunoadsorption (ABO-IA) with Glycosorb®, commonly used for desensitization therapy in ABO-incompatible living donor renal transplantation, specifically eliminates circulating isohemagglutinins and might represent a novel treatment option for post-HSCT PRCA. In this prospective observational (n = 3) and retrospective (n = 3) analysis of six adult HSCT-recipients with PRCA, ABO-IA was initiated at 159 (range: 104–186) days following HSCT. The median treatment frequency was 4.5 (range: 3.9–5.5) sessions/week. ABO-IA-treatment led to a continuous decrease in isohemagglutinin titers. Reticulocytes increased to ≥30 G/L after 17.5 (range: 4–37) immunoadsorption sessions over 28.5 (range: 6–49) days and continued to rise after that. By the end of the 3-month follow-up period after discontinuation of ABO-IA, all patients showed a sustained remission of PRCA and were independent of erythropoietin-stimulating agents and transfusions. No case of infection or graft-versus-host disease was observed. After a median follow-up of 22.03 (range: 6.08–149.00) months after ABO-IA-treatment, all patients were alive and showed a stable RBC engraftment of the donor blood group. Our data provide the first evidence for ABO-IA as an effective treatment for post-HSCT PRCA.
Highlights
Matching of human leukocyte antigen (HLA) alleles between donors and recipients is the primary determinant of successful allogeneic hematopoietic stem cell transplantation (HSCT)
Standard graft-versus-host disease (GVHD) prophylaxis consisted of cyclosporine A (CSA) + methotrexate (MTX) following myeloablative conditioning (MAC) or CSA + mycophenolate mofetil (MMF) following reducedintensity conditioning (RIC)
Our data demonstrate the successful resolution of Pure red cell aplasia (PRCA) in six major/bidirectional ABO-incompatible allogeneic HSCT recipients by ABO antigen-specific immunoadsorption (ABO-IA) using the Glycosorb R ABO immunoadsorption system
Summary
Matching of human leukocyte antigen (HLA) alleles between donors and recipients is the primary determinant of successful allogeneic hematopoietic stem cell transplantation (HSCT). The influence of ABO incompatibility on the overall outcome after HSCT appears to be negligible, several associated complications such as the delayed recovery of erythropoiesis, hemolysis, or pure red cell aplasia (PRCA) have to be recognized [2, 3]. The latter is related to the persistence of host B lymphocytes or plasma cells producing isohemagglutinins (IHAs) directed against donor red blood cell (RBC) antigens, leading to the disruption of erythroid hematopoiesis at the early precursor stage [2,3,4]. Apart from supportive measures including the administration of erythropoietin-stimulating agents (ESAs), other therapeutic approaches for PRCA include the modulation of immunosuppression, administration of immunosuppressive agents, or apheresis modalities like high-volume plasmaexchange (PE) or semi-selective immunoadsorption [5,6,7,8,9,10,11,12]
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