组织细胞坏死性淋巴结炎68例临床分析

目的 探讨组织细胞坏死性淋巴结炎(HNL)的临床特点,以提高对该病的认识.方法 回顾性分析北京大学第一医院1999-2009年住院治疗的68例HNL患者的病例资料,总结临床表现、实验室检查结果 及治疗和预后的特点.结果 HNL多见于年轻人,发病年龄平均(19±13)岁,男女性别比为1∶1.所有患者均有淋巴结肿大,65例(95.6%)伴发热,25例(36.8%)伴肝脾肿大,17例(25.0%)伴咽痛、咳嗽等呼吸道症状,10例(14.7%)伴一过性皮疹,35例(51.5%)伴不同程度外周血白细胞下降,17例(25.0%)伴不同程度肝功能异常.72.1%(44/61)血沉增高,11.1%(6/54)抗核抗体(ANA)阳性.淋巴结活检HE染色及免疫组化可见典型的病理学改变.34例(50.0%)患者应用了糖皮质激素治疗,疗程2周至3个月.7例(10.3%)患者复发,再次激素治疗仍有效.ANA阳性者中,1例合并系统性红斑狼疮(SLE),1例在2年后诊断为SLE.结论 HNL临床表现缺乏特异性,病因不清,易误诊,病理组织学及免疫组化检查是确诊的依据,治疗以糖皮质激素为主,预后良好,但易复发,且可合并或进展为SLE,应引起重视.

Clinical analysis of histiocytic necrotizing lymphadenitis in 68 cases

Objective To investigate and analyze the clinical features in patients with histiocytic necrotizing lymphadenitis (HNL). Methods A total of 68 HNL patients at our hospital between the years of 1999 to 2009 were enrolled. The clinical data were collected from the hospital records and the relevant literature was reviewed. Results HNL mainly affected young people with an average age of( 19 ± 13 ) years old and a female-to-male ratio of 1: 1. All patients had lymphadenectasis. And 95.6% patients (65 cases) were feverish and 36. 8% patients ( 25 cases ) had mild hepatosplenomegaly; 25.0% ( 17 cases ) upper respiratory symptoms such as sore throat and cough; 14. 7% (10 cases)skin rash in their history; 51.5% (35 cases) leucopenia; 25.0% ( 17 cases) hepatic dysfunctions; 72. 1% (44/61) elevated erythrocyte sedimentation rate (ESR); 11.1% (6/54) positive antinuclear antibody (ANA). The final diagnosis of HNL was confirmed by pathological examination and immunohistochemical staining of biopsy specimens. And 34 (50. 0% )patients received glucocorticoid for 2 weeks to 3 months. Seven ( 10. 3% ) patients relapsed in which glucocorticoid was effective. Of 6 patients with positive ANA, one case was complicated with systemic lupus erythematosus (SLE) and another case diagnosed with SLE at 2 years after HNL Conclusions The clinical manifestations of HNL lack specificity so that it can be easily misdiagnosed. While the etiology of HNL remains elusive, the histopathological examination of affected lymph nodes has contributed greatly to the final diagnoses of HNL. Glucocorticoid therapy is recommended for treatment. Generally, HNL has an excellent prognosis but it often relapses. It should be noted that HNL may coexist with SLE or evolve ultimately into SLE.