组织细胞坏死性淋巴结炎的研究进展

Progress Histiocytic Necrotizing Lymphadenitis

组织细胞坏死性淋巴结炎(HNL)是一种非特异性、非肿瘤性、自限性疾病。其发病率低,病因及发病机制不明,临床表现及实验室检查缺乏特异性,临床常表现为亚洲年轻女性不明原因发热,伴浅表淋巴结肿大及一过性白细胞减少,容易误诊。HNL诊断主要依靠淋巴结活检,病理改变以凝固性坏死及组织细胞增生为主,但由于其病理组织学多样性,仍存在误诊风险,故需要与多种疾病相鉴别。常规抗感染及抗结核治疗效果差,糖皮质激素治疗效果较好,大多数预后佳。然而,部分患者可进展为系统性红斑狼疮,两者之间的联系有待进一步研究。

Histiocytic necrotizing lymphadenitis （HNL） is a non-specific, non-tumor, self-limiting disease. The inci- dence of the disease is low, and the etiology and pathogenesis are unknown. The lack of specific clinical manifestations and laboratory tests may easily lead to misdiagnosis. The clinical manifestations of the disease include unknown cause of fever, superficial lymphadenopathy and a transient leukopenia in young women. HNL diagnosis mainly relies on lymph node biopsy. Pathological changes are mainly coagulation necrosis and tissue cell proliferation, however, due to its histopathologi- cal diversity,there are still some risks of misdiagnosis which needs to be differentiated from a variety of diseases. Conven- tional anti-infection and anti-tuberculosis treatment effect is poor, while glucocorticoid treatment is effective with generally favorable prognosis. However, since part of these patients may develop into systemic lupus erythematosus, the link between them needs to be further studied.