获得性噬血细胞综合征15例临床分析

陆静峰+张彦芳+高武

摘 要 目的：探讨获得性噬血细胞综合征（aHPS）的临床特点，为其临床诊断及治疗提供有效的参考资料。方法：回顧性分析15例aHPS患者的临床特点、实验室检查结果、治疗及预后。结果：15例患者均有发热，脾肿大，部分肝肿大、淋巴结肿大、肝肾功能损害、消化道出血症状。实验室检查均有不同程度的血细胞减少、肝功能异常，高甘油三脂血症、铁蛋白>1 000 ng/ml，部分NK细胞活性下降、低纤维蛋白原血症、细胞因子升高。骨髓检查可见噬血现象。通常采用积极治疗原发病、大剂量使用丙种球蛋白和糖皮质激素及依托泊苷的疗法。15例患者除1例失访外，均在1～6周内死亡。结论：aHPS病因以淋巴瘤居多，病情凶险，预后差，病死率极高，早期诊断，早期治疗，可望提高患者的生存率。

关键词 噬血细胞综合征 获得性 临床特点

中图分类号：R551.2 文献标识码：B 文章编号：1006-1533（2017）01-0055-04

Clinical characteristics of 15 patients with acquired hemophagocytic syndrome

LU Jingfeng1*， ZHANG Yanfang2， GAO Wu1**（1. Department of Hematology， Shanghai Putuo Hospital， Shanghai University of Traditional Chinese Medicine， Shanghai 200062， China；2. Department of Hematology， Shanghai Ninth Peoples Hospital affiliated to School of Medicine， Shanghai Jiao Tong University， Shanghai 201900， China）

ABSTRACT Objective： To investigate the clinical features of acquired hemophagocytic syndrome （aHPS） and provide an available reference for its clinical diagnosis and treatment. Methods： The clinical features， laboratory findings and outcomes of 15 patients with aHPS were retrospectively analyzed. Results： The fever and splenomegaly appeared in all patients while the clinical characteristics such as hepatomegaly， lymphadenopathy， the injury of liver and kidney and the gastrointestinal bleeding symptoms were found only in some patients. There were cytopenia， liver dysfunction， hypertriglyceridemia， serum ferritin >1 000 ng/ml， partly decrease of NK cell activity， hypofibrinogenemia and increase of cytokines to a certain degree in laboratory tests and hemophagocytosis was seen in bone marrow examinations in all patients. They were generally treated with high doses of gamma globulin and corticosteroids and etoposide and meanwhile their primary diseases were also actively treated. Fourteen cases of the patients died within 1～6 weeks in addition to one case of the lost follow-up. Conclusion： AHPS is mostly caused by lymphoma and is one of the dangerous diseases with extremely poor prognosis and high mortality. The survival rate of patients can be improved by early diagnosis and treatment.

KEY WORDS shemophagocytic syndrome； acquired； clinical characteristics

噬血细胞综合征（hemophagocytic syndrome，HPS）是由淋巴细胞和组织细胞异常活化，分泌大量细胞因子引起的一系列全身炎症性反应，有发热、脾肿大、铁蛋白升高及噬血现象等临床特征，病情凶险，预后极差；根据有无异常基因及继发因素，HPS主要分为遗传性HPS（genetic HPS）及获得性HPS（acquired hemophagocytic syndrome， aHPS）。本研究回顾性分析了15例aHPS患者的临床特征、实验室检查、治疗及预后，旨在进一步提高对本病的认识，为临床医生提供参考。

1 资料与方法

1.1 病例与诊断标准

收集上海交通大学医学院附属第九人民医院（北部）2009年8月至2015年8月间的住院患者，包括血液内科、风湿科、传染科、急诊科等，共15例aHPS患者。