Nagasawa M, Okawa H, Yata J
Department of Pediatrics, Faculty of Medicine, Tokyo Medical and Dental University, Japan.
Clin Immunol Immunopathol. 1991 Oct;61(1):18-28. doi: 10.1016/s0090-1229(06)80004-8.
A 4-year-old boy with pure red cell aplasia was investigated. Immunophenotypic analysis of peripheral blood lymphocytes revealed a marked increase of CD20+ cells, which fell from 25.9% in the active stage to 9.7% in remission. The plasma contained a suppressive activity against CFU-e and BFU-e formation by the patient's bone marrow cells, which disappeared when the disease went into remission. Prednisone (2 mg/kg/day) therapy was tried for 5 weeks, but produced no improvement. Subsequently, high-dose gamma-globulin therapy induced complete remission of anemia. A lymphoblastoid B cell line obtained from the patient before therapy produced a factor that suppressed erythropoiesis but not granulopoiesis. The suppressive activity resided in the immunoglobulin fraction and was adsorbed by an anti-immunoglobulin column. These results indicate that expansion of B cells producing an immunoglobulin which suppressed erythropoiesis was involved in the pathogenesis of the disease in this patient.
对一名患有纯红细胞再生障碍性贫血的4岁男孩进行了研究。外周血淋巴细胞的免疫表型分析显示CD20+细胞显著增加，其比例从活动期的25.9%降至缓解期的9.7%。血浆中含有针对患者骨髓细胞CFU-e和BFU-e形成的抑制活性，疾病缓解时该活性消失。尝试使用泼尼松（2mg/kg/天）治疗5周，但无改善。随后，大剂量丙种球蛋白治疗使贫血完全缓解。治疗前从患者获得的淋巴母细胞样B细胞系产生一种抑制红细胞生成但不抑制粒细胞生成的因子。抑制活性存在于免疫球蛋白部分，并被抗免疫球蛋白柱吸附。这些结果表明，产生抑制红细胞生成的免疫球蛋白的B细胞扩增参与了该患者疾病的发病机制。
