儿童噬血细胞综合征临床特征分析

Clinical features of hemophagocytic lymphohistiocytosis in children

目的探讨儿童噬血细胞综合征(HLH)的临床表现特征,影响HLH预后的危险因素及合理用药。方法回顾性分析23例HLH患儿的住院病历资料,并应用SPSS13.0统计软件进行统计分析。结果将患儿按预后分为好转组与恶化组进行比较,发现两组患儿在地塞米松的使用上差异性显著(P<0.05);将单因素分析筛选出的可能有意义的变量再进行Logistic回归分析,显示在本次研究中地塞米松是影响HLH预后的显著相关因子。对17例证实有EB病毒(EBV)感染的患儿进行分析,发现B淋巴细胞计数(CD19)及使用地塞米松对EBV-HLH预后的影响具有统计学意义(P<0.05)。结论儿童HLH的治疗过程中应注意继发感染及弥散性血管内凝血的出现,在严格按照HLH-04标准进行治疗的同时,是否存在仅用激素及对症治疗就可控制的"轻症"HLH有待进一步研究。

Objective To study the clinical features, risk factors and rational treatment of hemophagocytic lymphohistiocytosis （HLH） in children. Methods The medical data of 23 cases of HLH hospitalized in Children Hospital affiliated to Chongqing Medical University from Novem- ber 2004 to January 2010 were revicwed and their clinical features were analyzed. Results All these cases were divided into improved group and depraved group, we found that prominent difference revealed on application of dexamethasone between these two groups （ P 〈 0.05 ）. After analy- zing the data of 17 cases of EBV - HLH, we found that the number of B lymphocytes CD19 and application of dexamethasone can prominently in- fluence the prognosis of patients. Conclusion Clinical physicians should pay special attention to secondary infections and DIC occurred during the treatment course for HLH. The problem about ＂modest HLH＂ can be controlled by dexanlethasone alone or not needs further study.