中枢神经系统受累的噬血细胞淋巴组织细胞增生症预后分析  

Prognosis of the central nervous system involvement in patients with hemophagocytic lymphohistiocytosis

作　　者：文凤云 肖莉[1] 宪莹[1] 温贤浩[1] 管贤敏[1] 廖关羚 于洁[1] 

机构地区：[1]重庆医科大学附属儿童医院血液肿瘤科,400014

出　　处：《中华血液学杂志》2017年第10期848-852,共5页Chinese Journal of Hematology

摘　　要：目的了解中枢神经系统（cNs）受累的儿童噬血细胞淋巴组织细胞增生症（HLH）的临床特征及预后影响因素。方法回顾性分析2006年1月至2015年10月诊治的88例有CNS受累HLH患儿临床资料，采用Kaplan．Meier法绘制生存曲线，建立Cox回归模型对可能影响其预后的危险因素进行单因素及多因素分析。结果①88例CNS受累的HLH患儿中，男38例，女50例，中位年龄4岁1个月（6个月～15岁10个月），占同期诊治HLH患儿比例的57．9％（88／152）。②88例患儿中有54例进行脑脊液检查，其中20例（37．0％）脑脊液异常；有73例患儿进行头颅影像学检查，其中42例（57．5％）影像学异常。③88例患儿中3例失访，36例存活（1例继发癫痫），49例死亡。中位随访时间为37（6—122）个月，3年总体生存率为44％。④多因素分析结果显示脑脊液异常（HR=3．467，95％CI1．276—9．423，P=0．015）、未给予鞘注（HR：0．231，95％C10．081～0．657，P=0．006）是影响CNS—HLH患儿预后的独立危险因素。结论HLH患儿CNS受累率较高，预后差，少数还会遗留神经系统后遗症；脑脊液异常的患儿预后不良，积极的鞘注治疗可以改善预后。Objective To investigate the characteristics and prognostic factor of central nervous system （CNS） involvement in patients with hemophagocytic lymphohistiocytosis （HLH）. Methods From January 2006 to October 2015, 152 patients with HLH, 88 patients had CNS involvement, their clinical data were collected, and survival was analyzed using the Kaplan-Meier life table method, univariate and multivariate Cox regression model analyses were applied to identify the risk factors of prognosis. Results ① 57.9% patients complicated with neurological symptoms, cerebrospinal fluid abnormalities were observed in 37.0% patients, 57.5% patients had abnormal neuroradiology. ②36 patients survived well, 3 patients lost to follow-up, 49 dead, 1 survival patient had epilepsy. ③The 3-year overall survival rate of 88 patients was 44%. ④abnormal CSF and unreceived IT bore a significant, independent adverse prognostic value （P〈0.05）. Conclusion CNS involvement in HLH has a high frequency and poor prognosis, few patients remained neurologic sequelae; abnormal CSF related to poor prognosis, positive intrathecal injections could improve the prognosis.

关 键 词：淋巴组织细胞增多症 嗜血细胞性 中枢神经系统 预后 

分 类 号：R725.5[医药卫生—儿科]