儿童组织细胞坏死性淋巴结炎118例临床特点及预后分析

Clinical features and prognosis of 118 children with histiocytic necrotizing lymphadenitis

目的探讨儿童组织细胞坏死性淋巴结炎(HNL)的临床特点及预后。方法回顾性病例总结,分析2014年1月至2021年12月于首都儿科研究所附属儿童医院风湿免疫科首次诊断并治疗的118例HNL患儿的临床资料。分析其临床症状、实验室检查、影像学检查、病理结果、治疗及随访情况。结果118例患儿中男69例、女49例,发病年龄10.0(8.0,12.0)岁,范围为1.5~16.0岁。所有患儿均有发热及淋巴结肿大,74例(62.7%)存在血液系统受累、39例(33.1%)存在皮肤损伤。实验室检查主要表现为红细胞沉降率升高90例(76.3%)、血红蛋白降低58例(49.2%)、白细胞降低54例(45.8%)、抗核抗体阳性35例(29.7%)。97例(82.2%)患儿行淋巴结B超,均表现为颈部低回声结节样病灶,22例(18.6%)行颈部X线和(或)CT,7例(5.9%)行颈部磁共振成像。118例患儿均行淋巴结活检,病理结果均不支持淋巴瘤等恶性疾病及EB病毒感染,均提示为HNL。57例(48.3%)患儿未治疗自愈,61例(51.7%)予口服激素治疗,4例(3.4%)予吲哚美辛肛门给药。118例患儿随访时间为4(2,6)年,范围为1~7年,87例(73.7%)患儿发病1次且未发展为其他风湿免疫病,24例(20.3%)患儿存在不同程度复发。7例(5.9%)患儿出现多系统损伤,检测自身抗体均为中、高滴度阳性,其中5例患儿发展为系统性红斑狼疮、2例患儿发展为干燥综合征;7例患儿均给予口服激素治疗,其中6例患儿加用免疫抑制剂、2例患儿予以甲泼尼龙20 mg/kg冲击治疗。结论HNL首次发病部分可以自愈,对激素敏感,预后良好。对于反复发病且合并多系统损伤的HNL,随访时需监测抗核抗体滴度,注意其发展为其他风湿免疫病的可能,预后不佳。

Objective To explore the clinical features and prognosis of children with histiocytic necrotizing lymphadenitis(HNL).Methods The clinical data of 118 children with HNL diagnosed and treated in the Department of Rheumatology and Immunology of Children′s Hospital,Capital Institute of Pediatrics from January 2014 to December 2021 were retrospectively analyzed.The clinical symptoms,laboratory examination,imaging examination,pathological findings,treatment and follow-up were analyzed.Results Among the 118 patients,69 were males and 49 were females.The age of onset was 10.0(8.0,12.0)years,ranging from 1.5 to 16.0 years.All the children had fever lymph node enlargement,blood system involvement in 74 cases(62.7%),skin injury in 39 cases(33.1%).The main manifestations of laboratory examination were increased erythrocyte sedimentation rate in 90 cases(76.3%),decreased hemoglobin in 58 cases(49.2%),decreased white blood cells in 54 cases(45.8%)and positive antinuclear antibody in 35 cases(29.7%).Ninety-seven cases(82.2%)underwent B-mode ultrasound of lymph nodes,showing nodular lesions with low echo in the neck;22 cases(18.6%)underwent cervical X-ray and(or)CT;7 cases(5.9%)underwent cervical magnetic resonance imaging.Lymph node biopsy was performed in all 118 cases,and the pathological results did not support malignant diseases such as lymphoma or Epstein-Barr virus infection,suggesting HNL.Fifty-seven cases(48.3%)recovered without treatment,61 cases(51.7%)received oral steroid therapy,and 4 cases(3.4%)received indomethacin as anal stopper.The 118 cases were followed up for 4(2,6)years,ranging from 1 to 7 years,87 cases(73.7%)had one onset and did not develop into other rheumatological diseases,and 24 cases(20.3%)had different degrees of recurrence,7 cases(5.9%)had multiple system injuries,and all of the tested autoantibodies were positive for medium and high titers.All of them developed into other rheumatic immune diseases,among which 5 cases developed into systemic lupus erythematosus and 2 cases developed into Sjogren′s syndrome;7 cases were given oral steroid therapy,including 6 cases plus immunosuppressant and 2 cases receiving methylprednisolone 20 mg/kg shock therapy.Conclusions The first-onset HNL portion is self-healing,hormone-sensitive and has a good prognosis.For HNL with repeated disease and multiple system injury,antinuclear antibody titer should be monitored during follow-up,and attention should be paid to the possibility of developing into other rheumatological diseases,with poor prognosis.