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: Georgios Papakonstantinou, Helmut Loeffler, Torsten Haferlach, Wolfram Brugger
• Journal: European journal of haematology (Eur J Haematol) Vol. 84 Issue 6 Pg. 547-9 (Jun 2010) ISSN: 1600-0609 [Electronic] England
• PMID: 20113334 (Publication Type: Case Reports, Journal Article)

Chemical References

• Antibodies, Monoclonal
• Antibodies, Monoclonal, Murine-Derived
• Antigens, CD20
• Rituximab

Topics

• Adult
• Anemia, Hemolytic (blood, immunology, therapy)
• Antibodies, Monoclonal (therapeutic use)
• Antibodies, Monoclonal, Murine-Derived
• Antigens, CD20 (blood)
• Autoimmune Diseases (blood, immunology, therapy)
• Erythroblasts (immunology)
• Erythrocyte Aggregation (immunology)
• Humans
• Male
• Rituximab
• Treatment Outcome