成人噬血细胞性淋巴组织细胞增生症21例临床分析

Clinical analysis of 21 adult patients with hemophagocytic lymphohistocytosis

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。

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 January2005 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 183days（ 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.