背景:组织细胞坏死性淋巴结炎(HNL)是一种发病机制未知的自限性炎症性疾病。一小部分HNL患者可以发展噬血细胞淋巴组织细胞增生症(HLH),一种高炎症性疾病.这些患者被诊断为HNL伴HLH(HNL-HLH)。已经对儿科人群中的HNL-HLH进行了系统研究,然而,临床,实验室,成人HNL-HLH患者的放射学特征和结局仍有待探索。我们旨在探索临床,实验室,以及成人HNL-HLH患者的放射学特征和结局。
方法:收集2010年10月至2015年6月南京医科大学第一附属医院收治的HNL-HLH患者的临床资料。所有患者均行淋巴结活检,病理诊断为HNL。年龄,性别,临床表现,淋巴结体征,实验室发现和成像数据,并收集患者的病理结果。
结果:在这项研究中,我们报告了5例成人HNL-HLH患者。所有5例患者均表现为淋巴结肿大和持续发烧。实验室检查结果与HLH的诊断一致。18F-氟脱氧葡萄糖正电子发射断层扫描/计算机断层扫描(18F-FDGPET/CT)显示淋巴结肿大,FDG摄取增加,可能存在脾代谢亢进。所有患者对皮质类固醇反应良好,预后良好。在随访期间,五名患者中有两名被诊断为系统性红斑狼疮。
结论:我们的研究表明,患有HNL-HLH的成年患者表现出不同的临床,实验室,和放射学特征。预后良好,患者可以使用类固醇和支持性治疗。
BACKGROUND: Histiocytic necrotizing lymphadenitis (HNL) is a self-limited inflammatory disease of unknown pathogenesis. A very small fraction of patients with HNL could develop hemophagocytic lymphohistiocytosis (HLH), a hyperinflammatory disorder. These patients are diagnosed as HNL with HLH (HNL-HLH). HNL-HLH in the pediatric population has been systemically studied, however, the clinical, laboratory, and radiological features and outcomes of adult patients with HNL-HLH remain to be explored. We aimed to explore the clinical, laboratory, and radiological features and outcomes of adult patients with HNL-HLH.
METHODS: We collected the clinical data of patients with HNL-HLH admitted to the First Affiliated Hospital of Nanjing Medical University from October 2010 to June 2015. All the patients underwent lymph node biopsy and have a pathological diagnosis of HNL. The age, gender, clinical presentation, lymph node signs, laboratory findings and imaging data, and pathological findings of the patients were collected.
RESULTS: In this study, we reported five adult patients with HNL-HLH. All five patients showed enlarged lymph nodes and prolonged fever. Laboratory findings were consistent with the diagnosis of HLH. 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) showed enlarged lymph nodes with increased FDG uptake and splenic hypermetabolism could be present. All the patients responded well to corticosteroids and had a good prognosis. Two of the five patients were diagnosed with systemic lupus erythematosus during the follow-up.
CONCLUSIONS: Our study demonstrated that adult patients with HNL-HLH showed distinct clinical, laboratory, and radiological features. And the prognosis is good and patients could be managed with steroids and supportive care.