继发性嗜血细胞综合征15例临床分析

Clinical feature analysis of 15 cases with secondary hemophagocytic syndrome

目的探讨自身免疫病相关性与非自身免疫病相关性嗜血细胞综合征（HPS）两者的临床特征、治疗及预后。方法回顾性收集15例2008年1月至2010年6月我院风湿科及血液科住院的继发性嗜血细胞综合征患者，分析临床及实验室特征、治疗以及预后的关系。并比较自身免疫病相关性嗜血细胞综合征（A组）与非自身免疫病相关性嗜血细胞综合征（B组）之间的异同点。采用Fisher精确概率法、t检验和秩和检验。结果2组均表现出发热、出血、黄疸、肝脾肿大，A组特异性表现出关节痛、皮疹、自身抗体阳性，而B组中黄疸更常见（38％和100％，P=0．018）。但比较2组实验室指标及预后差异均无统计学意义（P〉0．05）激素联合免疫抑制剂和丙种球蛋白治疗预后更好（P〈0．05）。结论除黄疸外，自身免疫病相关性HPS与其他继发性HPS的临床表现及实验室指标无明显差异，激素联合免疫抑制剂及丙种球蛋白治疗有效。

Objective To investigate the clinical characteristics, treatment and prognosis of autoimmune diseases associated and non-autoimmune diseases associated hemophagocytic： syndrome. Methods Clinical records of 15 cases with secondary hemophagocytic syndrome＇ were collected and the relations with treatment and prognosis was analyze. The similarities and differences between autoimmune disease associated hemophagocytic syndrome （group A） and non-autoimmune disease associated hemophagocytic syndrome （group B） were compared. Fisher exact test, t test and Willcoxen test were used for statistical analysis. Results Both groups had fever, bleeding, jaundice, hepatosplenomegaly, and arthralgia, skin rash and positive of autoaptibodies in group A were discovered specifically. But in group B, the patients with icterus were more common （38% vs 100%, P=0.018）. There was no significant difference in their laboratory data and prognosis when compared between the two groups （P〉0.05）. The patients who received corticosteroids and IVIG and/or immunosuppressive agents had better prognosis （P〈0.05）. Conclusion Except for ieterus there is no significant difference in clinical features and laboratory data among autoimmune disease associated hemophagocytic syndrome and other secondary hemophagocytic syndrome. And the therapy with corticosteroids combined with IVIG and/or immunosuppressive agents is effective.