儿童朗格罕细胞组织细胞增生症合并中枢神经系统侵犯临床研究(附98例分析)

Clinical research of Langerhans cell histiocytosis combined with central nervous system involvement(analysis of 98 cases)

目的探讨儿童朗格罕细胞组织细胞增生症(LCH)合并中枢神经系统侵犯的高危因素、临床与影像学特点及预后。方法将首都医科大学附属北京儿童医院2005年1月至2008年12月收治的98例初发LCH患儿分3组进行神经系统症状、体征及头颅MRI检测,并定期监测上述指标以评估疗效,Ⅰ组为多系统危险组(57例),Ⅱ组为多系统低危组(19例),Ⅲ组为单纯骨破坏组(22例)。结果中枢神经系统受累患儿共24例,占24.5%,其中Ⅰ组17例,Ⅱ组7例。19例合并颅面骨破坏;16例出现神经系统症状,其中中枢性尿崩9例。头颅MRI显示受累部位为垂体者共14例次。对中枢受累患儿按LCH-Ⅲ方案联合化疗,目前处于非活动性疾病状态6例,疾病稳定状态10例,疾病进展4例,死亡4例。结论颅面骨破坏、合并危险器官受累是中枢神经系统发病的高危因素。最常见的影像学改变是下丘脑-垂体轴受累。早期及随诊中进行MRI监测非常重要。中枢神经系统一旦出现器质性病变常不可逆,严重影响患儿生活质量。

Objective To study risk factors, clinical and imaging features and prognosis of children LCH combined with central nervous system involvement. Methods We conducted neurological signs inspection, cranial MRI and periodical monitoring and assessment in 98 LCH patients in Bejing Children＇s Hospital, Capital Medical University during the period of 2005-2008. Then we use SPSS 13.0 software to analyze the log. Results （ 1 ） We found 24 patients having central nervous system involvement, 17 patients in group Ⅰ , 7 cases in group Ⅱ. （2）Totally 19 of them had craniofacial bone destruction, 16 of them had neurological symptoms and 9 had diabetes insipidus. Cranial MRI showed the involvement of pituitary was common and existed in 14 of them. （3） All of the 24 patients were treated by LCH- Ⅲ schema. From then on, 6 of them in non-active status, 10 of them in stable status, 4 of them in progressive status and 4 of them died. Conclusion Violation of CNS is a common combination of LCH. The risk factors of CNS include craniofacial bone destruction and ＂Risk organ＂ involvement. The most common imaging change is the hypothalamus-pituitary axis changes. MRI is a very important monitoring method. The central nervous system damaged is irreversible. Once occurring, it will significantly affect the life quality of patients.