Jin Jingjing, Lu Songjian, Lin Honghua
Department of Clinical Immunology, Anhui Provincial Children's Hospital, Hefei, China.
Front Pediatr. 2025 Aug 28;13:1638239. doi: 10.3389/fped.2025.1638239. eCollection 2025.
To investigate the clinical features, laboratory findings, treatment, and follow-up of pediatric histiocytic necrotizing lymphadenitis (HNL) to enhance understanding of this disease.
A retrospective analysis was conducted on clinical data of 44 pediatric patients diagnosed with histiocytic necrotizing lymphadenitis (HNL) who were admitted to our hospital from January 2018 to October 2024. The clinical features, laboratory findings, pathological results, treatment, and prognosis were examined.
44 patients, aged 4-14 years (mean age: 9.4 ± 2.6 years), including 28 males and 16 females, Among them, 41 patients (93.2%) were aged ≥6 years. All patients presented with fever and superficial lymphadenopathy. Other clinical manifestations comprised rash (10 cases, 22.7%), abdominal pain and vomiting (7 cases, 15.9%), hepatosplenomegaly (5 cases, 11.4%), weight loss (3 cases, 6.8%), and fatigue (3 cases, 6.8%). The clinical manifestations of HNL (e.g., fever, cervical lymphadenopathy) were nonspecific and overlapped with other pediatric conditions. Definitive diagnosis required lymph node biopsy, which consistently demonstrated HNL in all cases. Laboratory findings predominantly showed normal or reduced leukocyte counts (42 cases, 95.5%), elevated lactate dehydrogenase (LDH) (38 cases, 86.4%), increased erythrocyte sedimentation rate (ESR) (34 cases, 77.3%), and elevated D-dimer levels (28 cases, 63.6%). Lymph node ultrasound (36 cases, 81.8%) revealed hypoechoic nodules, while neck CT (7 cases, 15.9%) demonstrated nodular soft-tissue density shadows. Glucocorticoids were administered to 35 cases (79.5%). Two cases (4.5%) of secondary hemophagocytic lymphohistiocytosis (HLH) were treated with methylprednisolone pulse therapy or intravenous immunoglobulin. Three cases (6.8%) were administered glucocorticoids combined with disease-modifying antirheumatic drugs, 1 case (2.3%) received nonsteroidal anti-inflammatory drugs (NSAIDs) alone, and 8 cases (18.2%) resolved spontaneously without intervention. During a follow-up period ranging from 2 months to 6 years, no cases progressed to other rheumatic diseases, 5 cases (11.4%) experienced recurrence, whereas the other cases exhibited a satisfactory prognosis.
The clinical manifestations of HNL in pediatric patients are nonspecific, necessitating lymph node biopsy for definitive diagnosis. It is glucocorticoid-sensitive, and some cases may resolve spontaneously with a positive prognosis, but long-term monitoring is essential.
探讨儿童组织细胞坏死性淋巴结炎（HNL）的临床特征、实验室检查结果、治疗及随访情况，以增进对该疾病的了解。
对2018年1月至2024年10月我院收治的44例诊断为组织细胞坏死性淋巴结炎（HNL）的儿童患者的临床资料进行回顾性分析。检查其临床特征、实验室检查结果、病理结果、治疗及预后情况。
44例患者，年龄4 - 14岁（平均年龄：9.4±2.6岁），其中男性28例，女性16例，其中41例（93.2%）年龄≥6岁。所有患者均有发热和浅表淋巴结肿大。其他临床表现包括皮疹（10例，22.7%）、腹痛和呕吐（7例，15.9%）、肝脾肿大（5例，11.4%）、体重减轻（3例，6.8%）和乏力（3例，6.8%）。HNL的临床表现（如发热、颈部淋巴结肿大）无特异性，与其他儿科疾病重叠。明确诊断需要进行淋巴结活检，所有病例均确诊为HNL。实验室检查结果主要显示白细胞计数正常或降低（42例，95.5%）、乳酸脱氢酶（LDH）升高（38例，86.4%）、红细胞沉降率（ESR）升高（34例，77.3%）和D - 二聚体水平升高（28例，63.6%）。淋巴结超声检查（36例，81.8%）显示低回声结节，颈部CT检查（7例，15.9%）显示结节状软组织密度影。35例（79.5%）患者使用了糖皮质激素。2例（4.5%）继发性噬血细胞性淋巴组织细胞增生症（HLH）患者接受了甲泼尼龙冲击疗法或静脉注射免疫球蛋白治疗。3例（6.8%）患者使用了糖皮质激素联合改善病情抗风湿药物，1例（2.3%）患者单独使用非甾体类抗炎药（NSAIDs），8例（18.2%）患者未经干预自行缓解。在2个月至6年的随访期内，无病例进展为其他风湿性疾病，5例（11.4%）复发，其余病例预后良好。
儿童HNL的临床表现无特异性，需进行淋巴结活检以明确诊断。该病对糖皮质激素敏感，部分病例可自行缓解，预后良好，但长期监测至关重要。
