成人噬血细胞性淋巴组织细胞增生症21例临床分析

目的:探讨成人噬血细胞性淋巴组织细胞增生症(HLH)的临床特点。方法:对2005年1月－2015年1月在南京医科大学第二附属医院血液科收治的21例成人HLH患者的临床资料进行回顾性分析。结果:21例患者初治时均表现为高热、肝脾肿大、凝血功能异常、血常规三系或二系受累。病因分析中7例（33.3%）为血液肿瘤相关性；10例（47.6%）为感染，其中3例确诊为发热伴血小板减少综合征（新型布尼亚病毒核酸检测阳性）；1例（4.8%）肿瘤相关性（骨髓活检示转移癌）；3例（14.3%）原因不明。随访中死亡16例，存活3例（最长至今已5年7个月），2例失访；生存时间为4天～5.7年（中位生存时间183天）。结论:HLH为罕见的致死性疾病，成人更为少见。临床表现复杂，常伴有多脏器受损，病情凶险，进展迅速。预后大多不良。发病机制和治疗手段有待进一步研究。

Clinical analysis of 21 adult patients with hemophagocytic lymphohistocytosis

Objective:To investigate the clinical characteristics of adult patients with hemophagocytic lymphohistocytosis(HLH).Methods:The clinical and experimental data of 21 adult patients with HLH from Department of Hematology,The Second Affiliated Hospital of Nanjing Medical University,were collected retrospectively from January 2005 to January 2015.Results:All patients presented high fever,hepatosplenomegaly,hematostatic abnormality,and cytopenia at first visit.Seven cases(33.3%)were diagnosed as non-Hodgkins' lymphoma.10(47.6%) were diagnosed as infection,3 cases as fever with thrombocytopenia syndrome among them.1(4.8%) was malignancy-associated HLH,3(14.3%)without pathogenesis.Seventeen cases were dead during follow-up and two was still in follow-up(the longest was 5 years and 7 months till now).Two cases lost follow-up.The median survival time was 183 days(range from 4 days~5.7 years).Conclusion:HLH is an uncommon fatal disease and farely occurred in adults.The clinical presentation is complex,usually with multi-organ dysfunction,aggressive course and poor prognosis.The pathogenesis and treatment of HLH should be further studied.