Pure red cell aplasia after ABO-incompatible allogeneic stem cell transplantation in severe aplastic anemia with response to steroids: a case report and literature review

Abstract

Pure red cell aplasia (PRCA) is a rare complication after ABO-incompatible allogeneic stem cell transplantation, but its mechanism is still unknown. Here we report on a patient with severe aplastic anemia who developed PRCA after HLA-identical but major ABO-mismatched peripheral blood stem cell transplantation. Erythroid engraftment was successful with primary steroid treatment. We concluded that an adequate dose of steroids can be the first line of therapy for PRCA after ABO-mismatched allogeneic stem cell transplantation.

Prevention of pure red cell aplasia after major or bidirectional ABO blood group incompatible hematopoietic stem cell transplantation by pretransplant reduction of host anti-donor isoagglutinins

“…48,49 If this strategy is not successful, several other strategies have been proposed according to the pathophysiology of the disorder: plasmapheresis, 43,46,64 antithymocyte globulin, 38,41 erythropoietin, 39,42,44,61 corticosteroids, 53,56 rituximab, 47,54 and donorlymphocyte infusions to induce GvHD. 45,51 Virtually all of these treatments have only been evaluated in a few patients or in single case reports.…”

Response to high-dose dexamethasone for acquired pure red cell aplasia following ABO-mismatched allogeneic stem cell transplantation

“…3 Immunological changes due to withdrawal of cyclosporin A, 4 or development of GVHD, are followed by rapid resolution of delayed rbc engraftment. Many treatment options have been reported in the literature including high-dose EPO, 5 plasma exchange, immunoadsorption, antithymocyte globulin, Rituximab, 6 DLI, lowdose steroids 7 and bolus methylprednisolone. Evaluation of the efficacy of any treatment is difficult because of the eventual spontaneous resolution of PRCA post-transplantation.…”

Successful Treatment of Pure Red Cell Aplasia with Rituximab in Patients after ABO-Compatible Allogeneic Hematopoietic Stem Cell Transplantation

“…PRCA following ABO-incompatible allogeneic HSCT is associated with an interaction of recipient anti-A or anti-B isoagglutinins with donor erythroid precursors expressing A and/or B antigens [6]. Spontaneous remission has been noted but treatments such as plasma exchange, donor-derived leukocyte infusion, erythropoietin, and steroids may be necessary to avoid RBC transfusion and to decrease the risk of hemochromatosis [7, 8, 9, 10, 11]. …”