Hepburn Alastair L, Lampert Irvin A, Boyle Joseph J, Horncastle Donna, Ng W Fai, Layton Mark, Vyse Timothy J, Botto Marina, Mason Justin C
Department of Rheumatology, Imperial College London, UK.
Ann Rheum Dis. 2007 Aug;66(8):1106-9. doi: 10.1136/ard.2006.065003. Epub 2007 Feb 2.
An increase in leucocyte apoptosis and impaired clearance of apoptotic cells has been observed in patients with systemic lupus erythematosus (SLE). Apoptotic cells are likely to be a key source of autoantigens in SLE as they express many of the nuclear autoantigens (in surface blebs and apoptotic bodies) that are relevant to this disease. The clearance of apoptotic cells is usually a rapid process, such that few cells are usually seen in the extracellular environment in vivo. We report a case in which multiple apoptotic bodies were observed in the bone marrow of a patient with SLE that was complicated by an immune-mediated pancytopenia. We have subsequently examined the frequency of apoptotic cells, identified morphologically, and by caspase-3 staining in bone-marrow trephine samples taken from patients with SLE over a 10-year period of follow-up. A high proportion of bone marrows contained apoptotic debris. The novel demonstration of apoptotic bodies in vivo in patients with SLE is unusual and supports the notion that the marrow may be a target organ in the disease. Their abundance is also consistent with the hypothesis that normal clearance mechanisms are defective and/or overwhelmed in SLE.
系统性红斑狼疮（SLE）患者中已观察到白细胞凋亡增加及凋亡细胞清除受损。凋亡细胞可能是SLE自身抗原的关键来源，因为它们表达许多与该疾病相关的核自身抗原（存在于表面泡状结构和凋亡小体中）。凋亡细胞的清除通常是一个快速过程，因此在体内细胞外环境中通常很少见到细胞。我们报告了一例SLE患者，其骨髓中观察到多个凋亡小体，该患者并发免疫介导的全血细胞减少症。随后，我们在10年随访期间，对SLE患者骨髓活检样本中通过形态学鉴定及半胱天冬酶-3染色确定的凋亡细胞频率进行了检查。高比例的骨髓含有凋亡碎片。SLE患者体内凋亡小体的新发现并不常见，支持骨髓可能是该疾病靶器官的观点。它们的大量存在也与SLE中正常清除机制存在缺陷和/或不堪重负的假说一致。
