儿童组织细胞坏死性淋巴结炎18例分析

Clinical analysis of 18 children with histocytic necrotizing lymphadenitis

目的总结儿童组织细胞坏死性淋巴结炎的临床特征及治疗方案。方法回顾山东大学齐鲁儿童医院2020年2月至2021年2月期间依据淋巴结病理确诊的18例患儿的临床特点、治疗经过及预后,并与以往报道做比较,进一步总结该病的临床特征及治疗方案。结果除1例为复发病例外,其余患儿均为初发病例。所有患儿都有发热及淋巴结肿大,但并不是所有患儿都有淋巴结疼痛。少数患儿病程中会出现皮疹、关节疼痛、无菌性脑膜炎、冠状动脉和颈静脉扩张、无痛性手指关节肿胀等。大部分患儿使用糖皮质激素治疗后治愈,但有1例患儿病情多次反复,加用羟氯喹治疗后病情控制仍不理想,有进展为系统性红斑狼疮的风险。结论与以往研究相比,儿童发病率明显上升,应引起儿科医生的重视。该病可累及全身多个脏器及器官,冠状动脉和颈静脉扩展、无痛性手指关节肿胀为首次报道。为减少该病的复发及防止进展为系统性红斑狼疮必要时可延长糖皮质激素的使用疗程。

【Objective】To summarize the new clinical features and treatment of children with histocytic necrotizing lymphadenitis(HNL).【Methods】The clinical features,treatment and prognosis of 18 HNL children diagnosed by lymph node pathology from February 2020 to February 2021 were collected by review medical records.New clinical features and treatment of the children were summarized by comparing with the previous reports.【Results】Except for one relapsed case,the rests were all first onset cases.All children had fever and swollen lymph nodes,but not all children had painful lymph nodes.A few cases had rashes,joint pain,aseptic meningitis,coronary arteries and jugular veins dilation and painless finger joints swelling.Most of the c hildren were cured after treatment with glucocorticoids,but one case had multiple relapses,the disease control is still not ideal after treatment with hydroxychloroquine,and he had the possibility of progression to systemic lupus erythematosus.【Conclusion】Compared with the past,the morbidity rate of HNL in children had increased significantly,which should arouse the attention of pediatricians.The disease can affect multiple organs of the body.Coronary artery and jugular vein dilation,and painless finger joint swelling were first reported.In order to reduce the recurrence and prevent the progression to systemic lupus erythematosus of the disease,the use of glucocorticoids can be extended if necessary.