Pure red cell aplasia after major or bidirectional ABO incompatible hematopoietic stem cell transplantation: to treat or not to treat, that is the question

Abstract

Pure red cell aplasia (PRCA) is a complication related to major or bidirectional ABO mismatched hematopoietic stem cell transplantation. This disorder is characterized by anemia, reticulocytopenia, and the absence or virtual absence of erythroid progenitors, other causes such as infections, hemolysis, disease relapse, or drug toxicity having been excluded. Patients with PRCA may become RBC transfusion dependent for long periods, suffering an important long-term iron overload, alloimmunization, and transfusion reactions. The persistence of recipient isoagglutinins against donor ABO antigens produced by host residual plasmatic cells has been considered as the immunological cause of the prolonged erythroid aplasia. PRCA behaves in many cases as a self-limited condition and resolution may occur spontaneously within weeks, months, and even years. Many different therapeutic approaches have been reported for posttransplant PRCA as plasmapheresis, high doses of erythropoietin, donor lymphocyte infusions, anti-thymocyte globulin, Rituximab and steroids, among others. However, to date there is no standard of care and the question if patients with PRCA should be treated and at which point remains. The objective of this article is to review the natural evolution of PRCA, and the treatments that have been used over time focusing on their suitability and efficacy.