Successful treatment of pure red cell aplasia with a single dose of rituximab in a child after major ABO incompatible peripheral blood allogeneic stem cell transplantation for acquired aplastic anemia

Abstract

Summary:Pure red cell aplasia (PRCA) is a well-known although infrequent hematologic complication after allogeneic bone marrow transplantation. PRCA occurs in cases of major ABO-mismatch between donor and recipient and is believed to be due to inhibition of donor erythroid progenitors by residual host isohemagglutinins. We report a 10-year-old boy with post-hepatitis aplastic anemia (AA) who developed PRCA after HLA-matched familial peripheral blood stem cell transplantation (SCT) following conditioning with Cph 200 mg/kg + ATG 90 mg/kg. Granulocyte engraftment occurred on day +18, platelet engrafted on day +40, while reticulocytopenia at 0% persisted until day +118, and erythroid precursors were totally absent from bone marrow. After a single dose of rituximab 200 mg/m 2 administered on day +118 PRCA resolved and on day +132 the reticulocytes rose to 5.7%. On day +139 the Hb reached 137 g/l and the erythroid lineage in BM increased to 21%. We conclude that due to the rapid recovery from PRCA and lack of side effects, rituximab should be tried as firstline treatment of PRCA after allo-SCT. Bone Marrow Transplantation (2002) 30, 405-407. doi:10.1038/sj.bmt.1703668 Keywords: pure red cell aplasia; allogeneic stem cell transplantation; rituximab Pure red cell aplasia (PRCA) is an infrequent but wellrecognized complication of allogeneic bone marrow transplantation. Most cases of PRCA occur after transplantations with major donor-recipient ABO mismatch and are due to the long persistence of residual titers of natural recipient anti-A, 1 or very rarely, anti-B antibodies, 2 causing inhibition of donor erythroid progenitors. Additional risk factors include use of reduced intensity conditioning and immunosuppression with CsA in the post-transplant

Red blood cell-incompatible allogeneic hematopoietic progenitor cell transplantation

“…Case reports describing the treatment of patients with red cell aplasia after major ABO-incompatible transplantation have been reported. 27,81,91,92 Although this response is likely a result of depletion of B cells producing isoagglutinins, the mechanism of action and the overall efficacy of this treatment approach cannot be determined from these limited reports.…”

Successful anti-CD20 antibody treatment of pemphigus foliaceus after unrelated cord blood transplantation

“…6 Rituximab has been used in the treatment of many other autoimmune diseases, 7 including cytopenias occurring after HSCT. 8,9 Recently, anti-CD20 has been reported useful in the treatment of refractory chronic GVHD. 10 Taken together, these reports suggest that anti-CD20 should be considered early in the treatment of steroid-refractory GVHD and other more unusual immune-mediated complications after HSCT.…”

Pure red cell aplasia associated with parvovirus B19 infection occurring late after allogeneic bone marrow transplantation

“…Therapy for PRCA has included red blood cell transfusion, recombinant erythropoietin, steroids, androgens, rituximab, or various immunosuppressive drugs, including anti-thymocyte globulin, interferon, cyclophosphamide, azathioprine, methotrexate, and cyclosporine [17][18][19][20]. Manipulation of the immune system by changing immunosuppressive regimens in recipients of solid organ transplants or by treating HIV infection with highly active antiretroviral therapy has been anecdotally successful [21,22].…”

“…In bone marrow culture studies, the growth of burst forming units-erythroid (BFU-E) was at the low range of normal (16)(17)(18)(19)(20) at 16 per 100,000 marrow mononuclear cells (MMNC). Controls for this culture study were normal with colony forming units-granulocyte macrophage (CFU-GM) of 7 per 100,000 MMNC.…”