LYMPHOCYTES AND LMYPHOKINES IN APLASTIC ANEMIA

实验室和临床研究表明，再生障碍性贫血 贫血可能是由免疫介导的。在多中心 试验，150多名再生障碍性贫血患者和各种 血液学衰竭综合征患者进入一项抗心力衰竭对照试验 胸腺细胞球蛋白(ATG，Upjohn)。约50%的 急性重型再生障碍性贫血患者有血液学检查 改善，通常是到输液独立性，在三个月内 月份。10岁和28岁之间无显著差异 几天的治疗。慢性重度和中度患者 再生障碍性贫血对10天的ATG也有反应：患有 全血细胞减少症和细胞性骨髓病的表现相似。 然而，其他血液病包括骨髓纤维化， 阵发性睡眠性血红蛋白尿，纯红细胞再生障碍性贫血，以及 无核细胞性血小板减少，对ATG无效。我们 我也治疗过环孢素A的患者，这是一种具有更多 对T细胞功能的特异性影响。15例重症患者 ATG治疗失败的再生障碍性贫血患者接受 环孢素，先停用3个月，再停用3个月 强的松。有五个人回应了。所有患者在此期间均可康复。 环孢素和皮质类固醇的联合治疗 没有复发。接受较短疗程的五名患者 阿托伐他汀治疗前的环孢素A无反应。没有患者患有 钻石-布莱克凡综合征用环孢素A治愈，但 两名成人纯红细胞再生障碍性贫血患者中有一人 血液学完全缓解。环孢素A和ATG 似乎是治疗再生障碍性贫血的有效方法。最后， 初步数据表明，一种基本的免疫学 再生障碍性贫血的缺陷：白介素I(IL-L)的产生 再生障碍性贫血患者的单核细胞 慢性阻塞性肺疾病患者IL-L分泌减少，反之 骨髓纤维化是一种增生性疾病，明显 增加了。

Laboratory and clinical studies have suggested that aplastic anemia may be may immunologically mediated. In a multi-center trial, over 150 patients with aplastic anemia and a variety of hematologic failure syndromes entered a controlled trial of anti- thymocyte globulin (ATG, Upjohn). Approximately 50% of patients with acute severe aplastic anemia had hematologic improvement, usually to transfusion independence, within three months. There were no significant differences between 10 and 28 days of therapy. Patients with chronic severe and moderate aplastic anemia also responded to 10 days of ATG: patients with pancytopenia and cellular bone marrow behaved similarly. However, other hemotologic disorders including myelofibrosis, paroxysmal nocturnal hemoglobinuria, pure red cell aplasia, and amegakaryocytic thrombocytopenia, did not respond to ATG. We have also treated patients with cyclosporin A, an agent with more specific effects on T-cell function. Fifteen patients with severe aplastic anemia who had failed ATG therapy were treated with cyclosporin, for 3 months without and then for 3 months with prednisone. Five responded. All patients recover during the time of combined cyclosporin and corticosteroid therapy, and there have been no relapses. Five patients who received shorter courses of cyclosporin A prior to ATG did not respond. No patients with Diamond- Blackfan syndrome recovered with cyclosporin A, but one of two patients with adult pure red cell aplasia had a complete hemotologic remission. Cyclosporin A as well as ATG appears to be effective therapy in aplastic anemia. Finally, preliminary data has suggested a fundamental immunological defect in aplastic anemia: interleukin I (Il-l) production by monocytes from patients with aplastic anemia is markedly decreased, and, conversely, Il-l production in patients with myelofibrosis, a hyperproliferative disorder, is markedly increased.