成人噬血细胞综合征预后危险因素分析

Analysis of risk factors in adults with hemophagocytic syndrome

目的总结分析成人噬血细胞综合征的临床特征,探讨对其预后有影响的危险因素。方法回顾性分析30例临床诊断为噬血细胞综合征的患者的临床特征(临床特点、实验室检查及治疗)。比较死亡组与存活组患者的临床特点,并进行多因素Logistic回归分析,探讨患者死亡的危险因素,并计算OR值及OR的95%可信限(95%CI)。结果30例患者存活21例,死亡9例,病死率为30%。多因素Logistic回归分析提示,EB病毒感染、CD4/CD8比例倒置、血小板降低、Hb<30 g/L为死亡危险因素,相对危险度分别为5.421、4.816、3.823及1.097。结论噬血细胞综合征病因复杂,临床表现多样,病死率较高,早期诊断、早期化疗可提高患者的生存率。应重视并针对死亡的危险因素制订相应的防治措施,降低病死率。

Objective To investigate the clinical characteristics of adult patients with hemophagocytic syndrome （HPS）and possible risk factors for death. Methods A retrospective case control study was conducted on 30 adult with HPS, and their clinical features, laboratory data, treatments and prognosis factors were reviewed. Logistic regression analysis was performed to determine possible factors affecting prognosis, and to determined independent predictors for death. The odds rations （OR） and their 95% confidence intervals （95%C/） were estimated. Results 21 adult with HPS were alive, while 9 cases were dead. The incidence of death was 30%. By Logistic regression analysis, Epstein-Barr virus-infected （OR=5.421）, reverse of CD4/CD8 T cell （OR=4.816）, thrombocytopenia （OR=3.823） and Hb （OR=1.907） independently predicted death. Conclusion Adult HPS presented complicated causes, diverse clinical characteristics and high mortality. Recognize, diagnose an d treat it with HLH-2004 project as early as possible are the key to improve prognosis. More attention should be paid to deal with these risk factors and to prevent the development of serious complications associated with HPS.