上海交通大学学报（医学版）

›› 2010, Vol. 30 ›› Issue (10): 1300-.doi: 10.3969/j.issn.1674-8115.2010.10.028

• 短篇论著 • 上一篇    下一篇

成人嗜血细胞综合征19例临床分析

赵明哲1,2, 赵维莅1, 沈志祥1   

  1. 1.上海交通大学 |医学院附属瑞金医院血液科, 上海 200025； 2.浙江省金华市中心医院血液科, 金华 321000
  • 出版日期:2010-10-25 发布日期:2010-10-27
  • 通讯作者: 沈志祥, 电子信箱: drshenzx@yahoo.com。
  • 作者简介:赵明哲（1973—）, 男, 主治医师, 学士；电子信箱: doctorzmz@126.com。

Clinical analysis of 19 adult patients with hemophagocytic syndrome

ZHAO Ming-zhe1,2, ZHAO Wei-li1, SHEN Zhi-xiang1   

  1. 1.Department of Hematology, Ruijin Hospital, Shanghai Jiaotong University School of Medicine, Shanghai 200025, China|2.Department of Hematology, Jinhua Municipal Central Hospital, Jinhua 321000, China
  • Online:2010-10-25 Published:2010-10-27

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摘要：

目的 探讨成人嗜血细胞综合征（HPS）的临床特点。方法 对2005年1月—2009年1月在上海交通大学医学院附属瑞金医院血液科就诊的19例成人HPS患者的临床资料进行回顾性分析。结果 19例患者初治时均表现为高热、肝脾肿大、凝血功能异常、血像三系或二系受累。10例（52.6%）最终确诊为非霍奇金淋巴瘤，2例（10.5%）为感染，1例（5.3%）为急性淋巴细胞白血病，6例（31.6%）原因不明。随访中死亡14例，存活1例（至今已6个月），4例失访；生存时间为2~6个月（中位生存时间3个月）。结论 HPS为罕见的致死性疾病，成人更为少见；临床表现复杂，常伴有多脏器受损的表现，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。

关键词: 嗜血细胞综合征, 发病机制, 治疗\预后

Abstract:

Objective To investigate the clinical characteristics of adult patients with hemophagocytic syndrome (HPS). Methods The clinical and experimental data of 19 adult patients with HPS from Department of Hematology, Ruijin Hospital, Shanghai Jiaotong University School of Medicine, were collected retrospectively from January 2005 to January 2009.ResultsAll patients presented high fever, hepatosplenomegaly, hematostatic abnormality, and cytopenia at first visit. Ten cases (52.6%) were diagnosed as non-Hodgkin's lymphoma; 2 (10.5%), as infection; 1 (5.3%), as acute lymphoblastic leukemia; and 6 (31.6%) without known etiology. Fourteen cases were dead during follow-up and one was still in follow-up (6 months till now). Four cases lost follow-up. The median survival time was 3 months (range 2-6 months). Conclusion HPS is an uncommon fatal disease, and rarely occurred in adult population. The clinical presentation is complex, usually with multi-organ dysfunction, aggressive course, and poor prognosis. The pathogenesis and treatment of HPS should be further studied.

Key words: hemophagocytic syndrome, pathogenesis, treatment, prognosis