儿童组织细胞坏死性淋巴结炎47例病例系列报告OA

Background The incidence of histiocytic necrotizing lymphadenitis(HNL)in children has gradually increased in recent years.The clinical manifestations of this disease vary greatly individuals,and laboratory tests lack specificity,making it prone to misdiagnosis and missed diagnosis in the early stages.Objective To explore the clinical characteristics of children with HNL in order to improve understanding of this disease.Design Case series report.Methods Clinical data of pediatric patients with HNL admitted to Sun Yat-sen Memorial Hospital from January 2008 to December 2024 were retrospectively analyzed using the hospital's electronic medical records system.Main Outcome Measures Clinical manifestations,laboratory findings,treatment modalities,and prognostic outcomes.Results The cohort comprised 34 males(72.3%)and 13 females,with an onset age ranging from 2 to 14 years(median 10 years).All patients presented with lymphadenopathy,while fever was observed in 42 cases(89.4%),hematological involvement in 33(70.2%),hepatosplenomegaly in 28(59.6%),and allergic diseases in 13(27.6%).Laboratory abnormalities included elevated erythrocyte sedimentation rate in 34 cases(72.3%),leukopenia in 33(70.2%),and increased C-reactive protein in 20(42.5%).Additional findings included elevated serum total IgE levels in 22 cases(55.0%),positive serum Epstein-Barr virus(EBV)-DNA in 17 of 39 tested(43.6%),positive Mycoplasma pneumoniae antibodies in 15 of 37 tested(40.5%),and positive antinuclear antibodies(ANA)in 10 of 35 tested(28.6%).Corticosteroids were administered to 32 patients(68.1%),with 12(25.5%)receiving adjunctive immunosuppressive therapy,including leflunomide(n=9)and hydroxychloroquine(n=3).One patient experienced disease recurrence 2.5 years post-diagnosis,and three patients developed systemic lupus erythematosus(SLE).Conclusion HNL in pediatric populations primarily manifests in school-aged children,with a notable male predominance.The condition is frequently associated with infections caused by EBV and Mycoplasma pneumoniae,and a subset of cases presents concurrently with allergic disorders.Glucocorticoid therapy has demonstrated efficacy in treatment.In pediatric patients exhibiting disease recurrence,concurrent autoimmune manifestations,and positive ANA results,adjunctive immunosuppressive therapy demonstrates improved clinical outcomes.Those with persistent ANA positivity necessitate extended,rigorous monitoring to detect potential progression to additional autoimmune disorders.