Abstract：
OBJECTIVE: To study the bone marrow (BM) immunohistomorphological characteristics in adult systemic lupus erythematosus (SLE) associated macrophage activation syndrome (SLE-MAS).
METHODS: Immunohistochemical (IHC) expression of CD3, CD8, perforin (PFN), and CD163 was studied on BM trephine biopsies from 30 cytopenic adult SLE cases (male: female = 1:5, age; 24 years, range; 19-32) and compared them with ten age matched controls. Clinicopathological parameters were compared among the cases likely (L) or unlikely (U) to have MAS using probability scoring criteria. The best cut off laboratory parameters to discriminate between the two were obtained through receiver operator curve (ROC) analysis.
RESULTS: MAS occurred in 12/30 (40%) cases and was more commonly associated with prior immunosuppressive therapy (p = .07), ≥ 3 system involvement (p = .09), lower fibrinogen (p < .01), increased triglyceride (p = .002), increased BM hemophagocytosis (p = .002), and higher MAS score [185 (176-203) vs. 105 (77-119), p < .01] than MAS-U subgroup. Although PFN+CD8+ T lymphocytes significantly decreased among cases than controls (p < .05), it was comparable between MAS-L and MAS-U subgroups. Fibrinogen (< 2.4 g/L, AUC; 0.93, p < .01), hemophagocytosis score (> 1.5, AUC; 0.71, p = .03), and an MAS probability score of ≥ 164 (AUC; 1, p < .01) discriminated MAS from those without MAS.
CONCLUSIONS: We noted a decrease in perforin mediated CD8 + T cell cytotoxicity in SLE. Immunohistochemical demonstration of the same along with histiocytic hemophagocytosis on BM biopsy may be useful adjunct in early diagnosis and management of MAS in SLE.
METHODS: Immunohistochemical (IHC) expression of CD3, CD8, perforin (PFN), and CD163 was studied on BM trephine biopsies from 30 cytopenic adult SLE cases (male: female = 1:5, age; 24 years, range; 19-32) and compared them with ten age matched controls. Clinicopathological parameters were compared among the cases likely (L) or unlikely (U) to have MAS using probability scoring criteria. The best cut off laboratory parameters to discriminate between the two were obtained through receiver operator curve (ROC) analysis.
RESULTS: MAS occurred in 12/30 (40%) cases and was more commonly associated with prior immunosuppressive therapy (p = .07), ≥ 3 system involvement (p = .09), lower fibrinogen (p < .01), increased triglyceride (p = .002), increased BM hemophagocytosis (p = .002), and higher MAS score [185 (176-203) vs. 105 (77-119), p < .01] than MAS-U subgroup. Although PFN+CD8+ T lymphocytes significantly decreased among cases than controls (p < .05), it was comparable between MAS-L and MAS-U subgroups. Fibrinogen (< 2.4 g/L, AUC; 0.93, p < .01), hemophagocytosis score (> 1.5, AUC; 0.71, p = .03), and an MAS probability score of ≥ 164 (AUC; 1, p < .01) discriminated MAS from those without MAS.
CONCLUSIONS: We noted a decrease in perforin mediated CD8 + T cell cytotoxicity in SLE. Immunohistochemical demonstration of the same along with histiocytic hemophagocytosis on BM biopsy may be useful adjunct in early diagnosis and management of MAS in SLE.
摘要：
目的：研究成人系统性红斑狼疮（SLE）相关巨噬细胞活化综合征（SLE-MAS）的骨髓（BM）免疫形态学特征。
方法：CD3，CD8，穿孔素（PFN）的免疫组织化学（IHC）表达，和CD163在30例成人细胞减少性SLE患者的BM环钻活检中进行了研究（男性：女性=1：5，年龄；24岁，范围；19-32)，并将它们与10个年龄匹配的对照进行比较。使用概率评分标准比较可能（L）或不太可能（U）患有MAS的病例之间的临床病理参数。通过受试者操作曲线（ROC）分析获得区分两者的最佳截止实验室参数。
结果：MAS发生在12/30（40％）的病例中，并且与以前的免疫抑制治疗有关（p=.07），≥3个系统参与（p=.09），较低的纤维蛋白原（p<0.01），甘油三酯增加（p=0.002），BM吞噬作用增加（p=0.002），和更高的MAS得分[185（176-203）vs.105（77-119），p<.01]比MAS-U子组。尽管PFN+CD8+T淋巴细胞明显低于对照组(p<0.05),MAS-L和MAS-U亚组之间具有可比性.纤维蛋白原（<2.4g/L，AUC；0.93，p<0.01），吞噬作用评分(>1.5,AUC;0.71,p=.03),MAS概率评分≥164(AUC;1，p<.01)将MAS与没有MAS的区分开来。
结论：我们注意到SLE中穿孔素介导的CD8+T细胞毒性降低。同样的免疫组织化学证明以及BM活检上的组织细胞吞噬作用可能有助于SLE中MAS的早期诊断和治疗。
方法：CD3，CD8，穿孔素（PFN）的免疫组织化学（IHC）表达，和CD163在30例成人细胞减少性SLE患者的BM环钻活检中进行了研究（男性：女性=1：5，年龄；24岁，范围；19-32)，并将它们与10个年龄匹配的对照进行比较。使用概率评分标准比较可能（L）或不太可能（U）患有MAS的病例之间的临床病理参数。通过受试者操作曲线（ROC）分析获得区分两者的最佳截止实验室参数。
结果：MAS发生在12/30（40％）的病例中，并且与以前的免疫抑制治疗有关（p=.07），≥3个系统参与（p=.09），较低的纤维蛋白原（p<0.01），甘油三酯增加（p=0.002），BM吞噬作用增加（p=0.002），和更高的MAS得分[185（176-203）vs.105（77-119），p<.01]比MAS-U子组。尽管PFN+CD8+T淋巴细胞明显低于对照组(p<0.05),MAS-L和MAS-U亚组之间具有可比性.纤维蛋白原（<2.4g/L，AUC；0.93，p<0.01），吞噬作用评分(>1.5,AUC;0.71,p=.03),MAS概率评分≥164(AUC;1，p<.01)将MAS与没有MAS的区分开来。
结论：我们注意到SLE中穿孔素介导的CD8+T细胞毒性降低。同样的免疫组织化学证明以及BM活检上的组织细胞吞噬作用可能有助于SLE中MAS的早期诊断和治疗。
