title: 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: 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 first-line treatment of PRCA after allo-SCT.
authors.affiliation: Research Institute for Pediatric Hematology of the Russian Federation, Russian Children's Hospital, Moscow, Russian Federation.
authors.forename: A A
authors.initials: AA
authors.lastname: Maschan
authors.forename: E V
authors.initials: EV
authors.lastname: Skorobogatova
authors.forename: D N
authors.initials: DN
authors.lastname: Balashov
authors.forename: E D
authors.initials: ED
authors.lastname: Pashanov
authors.forename: P E
authors.initials: PE
authors.lastname: Trakhtman
authors.forename: I P
authors.initials: IP
authors.lastname: Schipitzina
authors.forename: Y V
authors.initials: YV
authors.lastname: Skvortsova
authors.forename: A G
authors.initials: AG
authors.lastname: Rumiantzev
mesh_terms: D000017:ABO Blood-Group System / Q000276:immunology; D000741:Anemia, Aplastic / Q000150:complications / Q000209:etiology / Q000628:therapy*; D000911:Antibodies, Monoclonal / Q000008:administration & dosage*; D058846:Antibodies, Monoclonal, Murine-Derived; D001787:Blood Group Incompatibility; D002648:Child; D018572:Disease-Free Survival; D006505:Hepatitis / Q000150:complications; D006801:Humans; D008297:Male; D036102:Peripheral Blood Stem Cell Transplantation / Q000009:adverse effects*; D012010:Red-Cell Aplasia, Pure / Q000188:drug therapy* / Q000209:etiology; D000069283:Rituximab; D014184:Transplantation, Homologous / Q000276:immunology; D016896:Treatment Outcome
chemical_list: D000017:ABO Blood-Group System; D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D000069283:Rituximab