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红细胞再生障碍与系统性红斑狼疮。

Erythrocyte aplasia and systemic lupus erythematosus.

作者信息

Kiely P D, McGuckin C P, Collins D A, Bevan D H, Marsh J C

机构信息

Department of Rheumatology, St. George's Hospital, London, UK.

出版信息

Lupus. 1995 Oct;4(5):407-11. doi: 10.1177/096120339500400512.

Abstract

Pure erythrocyte aplasia is a recognised feature of systemic lupus erythematosus (SLE); here we report two cases, one predating the onset of SLE, the other following a long period of disease quiescence. One case demonstrates the typical features of this disorder and was successfully treated with prednisolone. The second case is unusual in being resistant to immunosuppressive treatment. Bone marrow culture from the second patient revealed an inhibition of BFU-E colony formation in the presence of the patient's serum, indicating that a serum inhibitor of haemopoiesis was present. Furthermore, following T cell depletion of this patient's marrow, there was an increase in BFU-E, CFU-G and CFU-GM colony growth implicating, in addition, a possible T cell-mediated inhibition of marrow haemopoiesis. This is a novel observation and may explain the resistance shown by this patient to standard treatment.

摘要

纯红细胞再生障碍是系统性红斑狼疮(SLE)的一个公认特征;在此我们报告两例病例,一例在SLE发病之前,另一例在疾病长期静止之后。一例展现了这种病症的典型特征,并成功接受了泼尼松龙治疗。第二例不同寻常之处在于对免疫抑制治疗有抗性。对第二例患者的骨髓培养显示,在患者血清存在的情况下,爆式红系集落形成单位(BFU-E)集落形成受到抑制,表明存在一种造血血清抑制剂。此外,在对该患者的骨髓进行T细胞清除后,BFU-E、粒系集落形成单位(CFU-G)和粒-巨噬系集落形成单位(CFU-GM)集落生长增加,这还表明可能存在T细胞介导的骨髓造血抑制。这是一个新发现,可能解释了该患者对标准治疗表现出的抗性。

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