儿童组织细胞坏死性淋巴结炎的研究进展

Research progress of histiocytic necrotizing lymphadenitis in children

组织细胞坏死性淋巴结炎是一种罕见的、非肿瘤性自限性疾病,病因及发病机制不明。儿童患病人群以学龄期儿童较常见。临床上以发热、颈部淋巴结肿大为主要表现,其症状、体征及实验室检查缺乏特异性,常被误诊,确诊依赖细针穿刺细胞学检查或淋巴结活检病理组织学检查。多数患者采取对症支持治疗,少数患者需使用类固醇、免疫抑制及免疫支持治疗。本病中的罕见病例可伴有中枢或外周神经系统受累,严重者可并发多器官损伤,部分患儿有复发及继发自身免疫性疾病的风险,故应尽早诊断、避免过度诊疗,需长期随访监测复发。

Histiocytic necrotizing lymphadenitis is a rare,non-neoplastic and self-limiting disease with unknown etiology and pathogenesis.Children with the disease is more common in school-age children.It is characterized by fever and enlargement of lymph nodes in clinic.Its symptoms,signs and laboratory tests are nonspecific,so it is often misdiagnosed.Diagnosis of the disease depends on fine needle aspiration cytology or lymph node biopsy histopathology examinations.Most patients adopt symptomatic and supportive treatment.Few patients require steroids,immunosuppression and immune support therapy.In rare cases of this disease,the central or peripheral nervous system may be involved,multiple organ damage may occur in severe cases,and some child patients are at risk of relapse and secondary autoimmune diseases.Therefore,it should be diagnosed as early as possible to avoid excessive diagnoses and treatments.Long-term follow-up monitoring for recurrence is required.