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

发布时间：2018-10-24 22:30

【摘要】：目的:了解中枢神经系统(central nervous system,CNS)受累的儿童噬血细胞淋巴组织细胞增生症(hemophagocyic histiocytosis,HLH)的临床特点及预后影响因素。方法:2006年1月至2015年10月重庆医科大学附属儿童医院血液肿瘤中心诊治HLH患儿152例,88例有CNS受累,分析这88例患儿的临床病例资料,采用KM生存分析法绘制总体生存曲线,单因素及多因素COX回归模型分析影响其预后的危险因素。结果:1.2006年1月至2015年10月期间在我院诊治的152例HLH,88有CNS受累。88例CNS-HLH患儿中,73例为EBV-HLH(83%),2例CMV感染相关,8例为细菌感染相关;剩余5例未找到明确病因。2.59例(39%)患儿有神经症状体征,包括抽搐、意识改变、球麻痹、精神行为异常、共济失调、颈强直、角弓反张。20例(37%)患儿脑脊液淋巴细胞数增多或蛋白升高。42例(57.5%)患儿头颅影像学异常,包括脑沟加深增宽、脑萎缩、脑室周围白质异常、出血、钙化。3.88例CNS-HLH患儿中36例存活良好,3例失访,49例死亡,1例存活患儿继发癫痫;3年总体生存率为44%,中位生存时间4.5月。4.多因素COX回归分析提示脑脊液异常、鞘注是影响CNS-HLH预后的独立危险因素(P0.05)。结论:1.本组患儿以继发性HLH为主,CNS受累率较高;CNS-HLH预后差,死亡率高,少数还会遗留神经系统后遗症。2.脑脊液异常的患儿预后不良,积极的全身化疗+鞘注治疗对继发性HLH患儿CNS受累是有效的。
[Abstract]:Objective: to investigate the clinical features and prognostic factors of hemophagocytic lymphohistiocytosis (hemophagocyic histiocytosis,HLH) in children with central nervous system (central nervous system,CNS) involvement. Methods: from January 2006 to October 2015, 152 children with HLH were diagnosed and treated by the blood tumor center of affiliated Children's Hospital of Chongqing Medical University. 88 cases were involved with CNS. The clinical data of 88 cases were analyzed and the overall survival curve was drawn by KM survival analysis. Univariate and multivariate COX regression models were used to analyze the prognostic risk factors. Results: 1. From January 2006 to October 2015, there were 152 cases of HLH,88 with CNS involvement in our hospital. Among 88 cases of CNS-HLH, 73 cases were EBV-HLH (83%), 2 cases were related to CMV infection, 8 cases were related to bacterial infection, the remaining 5 cases had no definite etiology. 2.59 cases (39%) had neurological symptoms and signs. Including convulsions, changes in consciousness, bulbar paralysis, mental and behavioral disorders, ataxia, cervical ankylosis, angle arch backtension, 20 cases (37%) with increased cerebrospinal fluid lymphocyte count or increased protein, 42 cases (57.5%) with abnormal cephalic imaging, including deep widening of the sulcus, Brain atrophy, abnormal white matter around ventricle, hemorrhage and calcification. 36 cases survived well, 3 cases lost visit, 49 cases died and 1 case survived secondary epilepsy in 3.88 cases of CNS-HLH, the overall 3-year survival rate was 44 months and the median survival time was 4.5 months. Multivariate COX regression analysis showed that cerebrospinal fluid was abnormal and sheath injection was an independent risk factor for the prognosis of CNS-HLH (P0.05). Conclusion: 1. In this group, secondary HLH was the main cause, and CNS had a higher involvement rate, CNS-HLH had a poor prognosis and a high mortality rate, and a few patients still had neurological sequelae. 2. 2. The prognosis of children with abnormal cerebrospinal fluid is poor. Active systemic chemotherapy sheath injection is effective for CNS involvement in children with secondary HLH.
【学位授予单位】：重庆医科大学
【学位级别】：硕士
【学位授予年份】：2017
【分类号】：R725.5

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