title: Severe idiopathic erythroblastic synartesis: successful treatment with the anti-CD20 monoclonal antibody rituximab.
abstract: Erythroblastic synartesis is a very rare disorder, considered to be caused by autoimmune mechanisms, leading to aggregation of erythroid precursor cells in the bone marrow and subsequently to acquired dyserythropoiesis with severe, transfusion-dependent anemia. An association with lymphoproliferative or autoimmune diseases has been reported or strongly suggested in all six published cases. Here, we report a young patient with severe idiopathic erythroblastic synartesis without an underlying disease, who was successfully treated with rituximab, an anti-CD20 monoclonal antibody. The patient received rituximab at a dose of 375 mg/m(2) once weekly for 4 wk after failure of both immunosuppressive therapies with corticosteroids and intravenous immunoglobulins. At a follow-up of 30 months after treatment, the patient is still in continuous complete remission without any further treatment, suggesting that rituximab may induce prolonged remissions and eventually cure in this rare disease.
authors.affiliation: Medical Center, Department of Hematology-Oncology, Schwarzwald-Baar Clinic, Academic Teaching Hospital, University of Freiburg, Villingen-Schwenningen, Germany. gkonsta@nexgo.de
authors.forename: Georgios
authors.initials: G
authors.lastname: Papakonstantinou
authors.forename: Helmut
authors.initials: H
authors.lastname: Loeffler
authors.forename: Torsten
authors.initials: T
authors.lastname: Haferlach
authors.forename: Wolfram
authors.initials: W
authors.lastname: Brugger
mesh_terms: D000328:Adult; D000743:Anemia, Hemolytic / Q000097:blood* / Q000276:immunology / Q000628:therapy*; D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D058846:Antibodies, Monoclonal, Murine-Derived; D018951:Antigens, CD20 / Q000097:blood; D001327:Autoimmune Diseases / Q000097:blood* / Q000276:immunology / Q000628:therapy*; D004900:Erythroblasts / Q000276:immunology; D004903:Erythrocyte Aggregation / Q000276:immunology; D006801:Humans; D008297:Male; D000069283:Rituximab; D016896:Treatment Outcome
chemical_list: D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D018951:Antigens, CD20; D000069283:Rituximab