Medical Center, Department of Hematology-Oncology, Schwarzwald-Baar Clinic, Academic Teaching Hospital, University of Freiburg, Villingen-Schwenningen, Germany.
Eur J Haematol. 2010 Jun;84(6):547-9. doi: 10.1111/j.1600-0609.2010.01420.x. Epub 2010 Jan 22.
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.
成红细胞融合症是一种非常罕见的疾病，被认为是由自身免疫机制引起的，导致红系前体细胞在骨髓中聚集，随后出现获得性红细胞生成异常，导致严重的、依赖输血的贫血。在所有已发表的 6 例病例中，均报道或强烈提示存在与淋巴增生性或自身免疫性疾病的关联。在此，我们报告一例年轻的特发性成红细胞融合症患者，无潜在疾病，经抗 CD20 单克隆抗体利妥昔单抗治疗后获得成功。该患者在接受皮质类固醇和静脉注射免疫球蛋白两种免疫抑制治疗失败后，每周接受利妥昔单抗 375mg/m2，共 4 周。治疗后 30 个月的随访中，患者仍处于持续完全缓解状态，无需进一步治疗，表明利妥昔单抗可能在这种罕见疾病中诱导长期缓解并最终治愈。
