Severe idiopathic erythroblastic synartesis: successful treatment with the anti-CD20 monoclonal antibody rituximab.
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.
Papakonstantinou
Georgios
G
Medical Center, Department of Hematology-Oncology, Schwarzwald-Baar Clinic, Academic Teaching Hospital, University of Freiburg, Villingen-Schwenningen, Germany. gkonsta@nexgo.de
Loeffler
Helmut
H
Haferlach
Torsten
T
Brugger
Wolfram
W
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
D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D018951:Antigens, CD20; D000069283:Rituximab