【摘要】：目的:了解中樞神經(jīng)系統(tǒng)(central nervous system,CNS)受累的兒童噬血細(xì)胞淋巴組織細(xì)胞增生癥(hemophagocyic histiocytosis,HLH)的臨床特點(diǎn)及預(yù)后影響因素。方法:2006年1月至2015年10月重慶醫(yī)科大學(xué)附屬兒童醫(yī)院血液腫瘤中心診治HLH患兒152例,88例有CNS受累,分析這88例患兒的臨床病例資料,采用KM生存分析法繪制總體生存曲線,單因素及多因素COX回歸模型分析影響其預(yù)后的危險(xiǎn)因素。結(jié)果:1.2006年1月至2015年10月期間在我院診治的152例HLH,88有CNS受累。88例CNS-HLH患兒中,73例為EBV-HLH(83%),2例CMV感染相關(guān),8例為細(xì)菌感染相關(guān);剩余5例未找到明確病因。2.59例(39%)患兒有神經(jīng)癥狀體征,包括抽搐、意識(shí)改變、球麻痹、精神行為異常、共濟(jì)失調(diào)、頸強(qiáng)直、角弓反張。20例(37%)患兒腦脊液淋巴細(xì)胞數(shù)增多或蛋白升高。42例(57.5%)患兒頭顱影像學(xué)異常,包括腦溝加深增寬、腦萎縮、腦室周圍白質(zhì)異常、出血、鈣化。3.88例CNS-HLH患兒中36例存活良好,3例失訪,49例死亡,1例存活患兒繼發(fā)癲癇;3年總體生存率為44%,中位生存時(shí)間4.5月。4.多因素COX回歸分析提示腦脊液異常、鞘注是影響CNS-HLH預(yù)后的獨(dú)立危險(xiǎn)因素(P0.05)。結(jié)論:1.本組患兒以繼發(fā)性HLH為主,CNS受累率較高;CNS-HLH預(yù)后差,死亡率高,少數(shù)還會(huì)遺留神經(jīng)系統(tǒng)后遺癥。2.腦脊液異常的患兒預(yù)后不良,積極的全身化療+鞘注治療對繼發(fā)性HLH患兒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.
【學(xué)位授予單位】：重慶醫(yī)科大學(xué)
【學(xué)位級(jí)別】：碩士
【學(xué)位授予年份】：2017
【分類號(hào)】：R725.5

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