32例成人继发性噬血细胞综合征临床特点和预后分析

目的 探讨成人继发性噬血细胞综合征(HLH)临床特征和预后因素。 方法 收集32例成人继发性HLH患者临床资料,其中男17例(53%),女15 例(47%),19~88岁,中位年龄57岁。回顾性分析HLH患者的临床特点、病因及复发,采用单因素和Cox多因素分析预后因素。 结果 32例发热和血清铁蛋白升高,26例肝脾肿大,25例血细胞减少。病因分析中恶性肿瘤相关14例(43.75%),感染相关10例(31.25%),其他8例(25%)。32例最终死亡24例(75%),中位生存期27 d,复发率11.10%。单因素方差分析示,年龄、皮疹、血红蛋白、甘油三酯、凝血时间、活化部分凝血酶原时间与死亡相关(P<0.05),Cox多因素分析显示,年龄、甘油三酯水平是预后的独立危险因素。 结论 成人继发性HLH病因和临床特点多样,高龄和高甘油三酯是其死亡相关的独立预后因子。

Clinical features and prognosis of adult secondary hemophagocytic syndrome: an analysis of 32 cases

Objective To investigate the clinical features and prognostic factors of adult secondary hemophagocytic lymphohistiocytosis(HLH). Methods Clinical data of 32 cases of adult secondary HLH were collected, clinical features, etiology and recurrence were reviewed, and prognostic factors were analyzed with univariate and Cox multivariate analyses. Results Of the 32 patients, 17(53%)were male and 15(47%)were female, median age 57(19-88)years. Fever and elevated serum ferritin were observed in 32 cases, hepatosplenomegaly in 26 cases, and cytopenia in 25 cases. Etiology analyses showed that 14 cases(43.75%)were caused by malignant tumors, 10(31.25%)by infections, and 8(25%)by other factors. Ultimately 24(75%)deaths occurred. The median survival was 27 days and recurrence rate was 11.1%. One-way analysis of variance showed that age, rash, hemoglobin, triglyceride, clotting time and activated partial prothrombin time were related to death(P<0.05). Cox multivariate analysis indicated that age and triglyceride level were independent risk factors for prognosis. Conclusion Secondary HLH in adults has diverse etiology and clinical features. Advanced age and high triglyceride level are independent prognostic factors associated with death.