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自体造血干细胞移植治疗伴有严重肺损伤的难治性系统性红斑狼疮:一例报告

Autologous blood stem cell transplantation in refractory systemic lupus erythematosus with severe pulmonary impairment: a case report.

作者信息

Brunner M, Greinix H T, Redlich K, Knöbl P, Smolen J, Leitner G, Derfler K, Graninger W, Kalhs P, Machold K

机构信息

Internal Medicine I, University of Vienna Medical School, Waehringer Guertel 18-20, A-1090 Vienna, Austria.

出版信息

Arthritis Rheum. 2002 Jun;46(6):1580-4. doi: 10.1002/art.10282.

Abstract

For patients with severe forms of autoimmunity, including systemic lupus erythematosus (SLE), purging autoreactive T cells from the immune repertoire by transplanting autologous hematopoietic stem cells (ASCT) is a therapeutic option. We describe an 18-year-old woman with SLE who had been treated with corticosteroids, azathioprine, cyclophosphamide (CYC), and immunopheresis for 4 years, during which time mechanical ventilation for lupus pneumonitis had been repeatedly required. After the patient was conditioned by administration of CYC and antithymocyte globulin, a total of 8.87 x 10(6) purified CD34+ cells per kg of body weight was infused. Hematopoietic regeneration was observed within 9 days. Twenty-one months after ASCT, the patient continues to be in complete clinical remission, with no signs of SLE-related disease activity and without any immunosuppressive medications. Her pulmonary function has returned to normal. Although a longer followup is required for assessment of the durability of response, the patient's course indicates that ASCT may be a way to reinduce tolerance in patients with SLE.

摘要

对于患有严重自身免疫性疾病的患者,包括系统性红斑狼疮(SLE),通过移植自体造血干细胞(ASCT)清除免疫库中的自身反应性T细胞是一种治疗选择。我们描述了一名18岁的SLE女性患者,她接受皮质类固醇、硫唑嘌呤、环磷酰胺(CYC)和免疫吸附治疗4年,在此期间因狼疮性肺炎多次需要机械通气。在患者接受CYC和抗胸腺细胞球蛋白预处理后,每公斤体重共输注8.87×10⁶个纯化的CD34⁺细胞。9天内观察到造血再生。ASCT后21个月,患者持续完全临床缓解,无SLE相关疾病活动迹象,且未服用任何免疫抑制药物。她的肺功能已恢复正常。尽管需要更长时间的随访来评估反应的持久性,但患者的病程表明ASCT可能是一种使SLE患者重新诱导免疫耐受的方法。

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