title: Severe autoimmune hemolytic anemia after unrelated umbilical cord blood transplant for familial hemophagocytic lymphohistiocytosis: significant improvement after treatment with rituximab.
abstract: A 4-month-old girl diagnosed with familial hemophagocytic lymphohistiocytosis underwent a matched unrelated, umbilical cord blood transplant. Six weeks later she developed severe acute autoimmune hemolytic anemia and thrombocytopenia requiring multiple transfusions. This was refractory to high-dose steroid and intravenous immunoglobulin, but did respond to Rituximab (anti-CD20 monoclonal antibody) 375 mg/m2. Hemolysis recurred after steroid tapering but responded to a second course of Rituximab. This case report highlights the difficulty in managing posttransplant autoimmune hemolytic anemia.
authors.affiliation: Department of Pediatrics, University of Iowa Hospitals and Clinics, Iowa City, IA 52242, USA. mohamed-radhi@uiowa.edu
authors.forename: Mohamed
authors.initials: M
authors.lastname: Radhi
authors.forename: Steve
authors.initials: S
authors.lastname: Rumelhart
authors.forename: David
authors.initials: D
authors.lastname: Tatman
authors.forename: Fred
authors.initials: F
authors.lastname: Goldman
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000744:Anemia, Hemolytic, Autoimmune / Q000188:drug therapy* / Q000209:etiology*; D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D058846:Antibodies, Monoclonal, Murine-Derived; D036101:Cord Blood Stem Cell Transplantation / Q000009:adverse effects*; D005260:Female; D005312:Fetal Blood / Q000637:transplantation; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007155:Immunologic Factors / Q000627:therapeutic use*; D007223:Infant; D051359:Lymphohistiocytosis, Hemophagocytic / Q000628:therapy*; D000069283:Rituximab; D013921:Thrombocytopenia / Q000188:drug therapy / Q000209:etiology
chemical_list: D000305:Adrenal Cortex Hormones; D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors; D000069283:Rituximab