儿童组织细胞坏死性淋巴结炎并中枢神经系统损害4例

Histiocytic necrotizing lymphadenitis combined with central nervous system damage in 4 children

目的总结儿童组织细胞坏死性淋巴结炎（HNL）合并中枢神经系统损害的临床特点。方法回顾性分析2009年1月至2014年12月在北京儿童医院住院诊治的4例HNL合并中枢神经系统损害病例的临床资料，并复习总结PubMed及CNKI等收录的12例患儿的文献资料。结果4例患儿以发热、淋巴结大起病，头痛3例，意识改变2例，抽搐1例；脑脊液改变与病毒性脑炎相似；2例头颅磁共振成像出现脑实质损害；脑脊液病原学检查2例EB病毒壳抗原（EBV—VCA）-IgM阳性，2例无阳性发现；淋巴结活检均符合HNL典型改变，1例EBV编码的RNA（EBER）检测阳性。文献复习中12例患者经淋巴结活检确诊HNL，中枢神经系统损害表现可有头痛、意识障碍。无病原学阳性发现。头颅影像受累部位可有颞叶、海马、尾状核、软脑膜等。结论HNL可以合并中枢神经系统损害，表现为无菌性脑膜炎、脑炎等，本病多数预后良好，少数可有神经系统后遗症，可复发，有必要长期随诊。EBV在HNL合并中枢神经系统损害的作用机制尚需进一步研究。

Objective To summarize the clinical features of histiocytic necrotizing lymphadenitis （HNL） and the lesion of central neural system in children. Methods Data of the 4 cases in Beijing Children＇s Hospital from January 2009 to December 2014 were reviewed and analyzed, as well as other files of 12 patients from PubMed, CNKI, etc. Results All 4 patients mentioned above started from fever and lymphadenopathy,3 cases had headache,2 cases of them experienced altered mental status, and 1 case had eclampsia. The findings of cerebrospinal fluid （CSF） test was similar to viral encephalitis ;2 cases had normal magnetic resonance imaging （MRI） result;1 case with MRI test indica- ted brain white matter diseases with extensive intracerebral hemorrhage, and 1 case had small range of encephalomalacia in left cerebral hemisphere and cerebral dura mater. Through CSF examination ,2 cases were positive to Epstein - Barr viral capsid antigen （ EBV - VCA） - IgM ,while the 2 other cases had no positive trace of EBV - VCA - IgM. Lymph node biopsy was in accordance with HNL typical changes, 1 case was positive for Epstein - Barr virus encoded RNA （EBER）. Twelve cases in previous literatures were diagnosed as HNL by lymph node biopsy, and their clinical features of central nervous system were headache and altered mental status. There was no change in pathogen. It is also found that the cases in which CSF was performed had shown being negative. CT/MRI image descriptions involved in temporos- phenoid lobe,hippocampus, caudate lobe, leptomeninges, etc. Conclusions HNL can be complicated with lesions of the central nervous system, characterized by aseptic meningitis, encephalitis, etc. The prognosis of most cases is good, a few can have neurological sequelae. HNL may relapse, so long - term follow - up is ne-cessary. Epstein - Barr virus in HNL and lesion of central nervous system mechanism still needs further research.