儿童组织细胞坏死性淋巴结炎21例临床及免疫病理特征分析

Analysis of clinical and pathological features of histiocytic necrotizing lymphadenitis in 21 children

目的探讨儿童组织细胞坏死性淋巴结炎(HNL)的临床、病理及免疫组化特点,以进一步提高对该病的认识,减少误诊。方法回顾性分析21例组织细胞坏死性淋巴结炎的临床资料。结果所有患者均有发热、淋巴结肿大,16例白细胞减少,淋巴结活检可见不同程度的凝固性坏死伴多种形态的组织细胞、淋巴细胞浸润,免疫组化染色显示组织细胞及T细胞较多,很少见到B细胞。14例经激素治疗,疗效显著,随访3年,3例复发,1例发展为系统性红斑狼疮,1例发展为强直性脊柱炎。结论 HNL临床表现无特异性,淋巴结活检可明确诊断,糖皮质激素治疗有效,预后多良好,部分有复发,需长期随访。

Objective To investigate the clinical, pathological, and characteristics of histiocytic necrotizing lym- pbadenitis（NHL） in children for improving the understanding of the disease and avoiding the misdiagnosis. Methods The chnical data of 21 patients with histiocytic necrotizing lymphadenitis were retrospectively analyzed. Results All patients had fever and lymph node enlargement. Leukopenia was observed in 16 patients. The lymph node biopsy showed different degree of coagulative necrosis with various forms of invasive histiocytes and lymphocytes, and the immunohistochemical staining revealed many histiocytes and T cells, but less B cells. Forteen patients were administrated with glueocorticoid and benefited significantly. After 3 years of follow-up, 3 cases re- curred, one patient developed systemic lupus erythematosus, and one patient developed ankylosing spondylitis. Conclusion HNL has no specific clinical manifestations, and can be confirmed by lymph node biopsy. Glcocorticoid therapy is effective. Most of them have a good prognosis, but some cases recur, so the long-term follow-up is necessary.