Natural killer cell dysfunction in patients with systemic-onset juvenile rheumatoid arthritis and macrophage activation syndrome
Natural killer cell dysfunction in patients with systemic-onset juvenile rheumatoid arthritis and macrophage activation syndrome
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DOI：
10.1067/mpd.2003.110
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发表时间：
2003-03-01
影响因子：
5.1
通讯作者：
Filipovich, A
中科院分区：
文献类型：
--
作者：
Grom, AA;Villanueva, J;Filipovich, A
目的探讨系统性幼年类风湿关节炎(SoJRA)合并巨噬细胞活化综合征(MAS)患者的自然杀伤细胞(NK)和细胞毒功能。方法采用流式细胞术检测NK细胞(CD56+/TCRaP-)、NK T细胞(CD56+/TCRalphabeta+)和CD8+细胞的穿孔素表达。将单个核细胞与NK敏感的K562细胞共孵育，检测NK细胞杀伤活性。7例患者中有4例NK活性降低，NK细胞数量减少，CDS+和CD56+细胞毒细胞穿孔素表达水平轻度升高。然而，其余三名MAS患者在所有细胞毒细胞群中NK活性降低，并伴有低水平的穿孔素表达，这种模式与穿孔素缺乏的家族性噬血细胞淋巴组织细胞增生症携带者的模式没有区别。结论NK功能障碍是SoJRA家族性噬血细胞淋巴组织细胞增生症和MAS共同的免疫学异常。SoJRA中NK细胞异常的程度有待进一步研究。
Objectives To assess natural killer (NK) and cytotoxic functions in patients with systemic-onset juvenile rheumatoid arthrithis (soJRA) complicated by macrophage activation syndrome (MAS).Methods NK cells (CD56+/TCRaP-), NK T cells (CD56+/TCRalphabeta+) and CD8+ cells were assessed for perforin expression by flow cytometry. NK cytotoxic activity was measured after coincubation of mononuclear cells with an NK-sensitive K562 cell line.Results Two major patterns of immunologic abnormalities were detected. Four of 7 patients had decreased NK activity, low NK cell numbers, and mildly increased levels of perforin expression in CDS+ and CD56+ cytotoxic cells. Three remaining patients with MAS, however, had decreased NK activity associated with low levels of perforin expression in all cytotoxic cell populations, a pattern indistinguishable from that in carriers of perforin-deficient familial hemophagocytic lymphohistiocytosis. Remarkably, two of these patients had previous episodes of MAS.Conclusions NK dysfunction is an immunologic abnormality common to both familial hemophagocytic lymphohistiocytosis and MAS of soJRA. The extent of NK cell abnormalities in soJRA needs to be further investigated.