基础医学与临床 ›› 2010, Vol. 30 ›› Issue (2): 189-193.

• 临床园地 • 上一篇    下一篇

噬血细胞综合征45例临床分析

马杰 郑文洁 张烜 唐福林   

  1. 中国医学科学院北京协和医学院北京协和医院 中国医学科学院北京协和医学院北京协和医院 中国医学科学院 北京协和医学院 北京协和医院 风湿免疫科 中国医学科学院 中国协和医科大学 北京协和医院 风湿免疫科
  • 收稿日期:2008-12-26 修回日期:2009-06-15 出版日期:2010-02-05 发布日期:2010-02-05
  • 通讯作者: 郑文洁

Clinical Analysis of 45 cases of Hemophagocytic Syndrome

Jie MA, Wen-jie ZHENG, Xuan ZHANG, Fu-lin TANG   

  1. PUMC Hospital, CAMS & PUMC PUMC Hospital, CAMS & PUMC Department of Rheumatology,PUMC Hospital,CAMS&PUMC Department of Rheumatology,PUMC Hospital,CAMS&PUMC
  • Received:2008-12-26 Revised:2009-06-15 Online:2010-02-05 Published:2010-02-05
  • Contact: Wen-jie ZHENG,

摘要: 目的 探讨噬血细胞综合征(HPS)患者的临床特征。方法 回顾性分析45例HPS患者的病因、临床及实验室检查特征、治疗反应及转归。结果 诱因:单纯病毒感染9例,结核感染2例,自身免疫性疾病相关4例,继发于恶性肿瘤22例,病因不明8例。临床主要表现为高热(93.3%)、肝肿大(77.8%)、脾肿大(80%)、淋巴结肿大(55.6%)、皮疹(24.4%)、消化道出血 (22.2%)、肾脏(35.6%)和神经系统受累(15.6%),并发弥漫性血管内凝血5例(DIC)(11.1%)。实验室检查表现为不同程度的血细胞减少(100%)、肝功异常(77.8%)、高三酰甘油(71.1%)、低纤维蛋白原血症(62.8%)、铁蛋白>500μg/L(87.5%)和NK细胞活性降低(92.9%)。骨髓检查均可见成熟的单核巨噬细胞吞噬血细胞现象。积极治疗原发病以及经糖皮质激素、免疫抑制剂、人免疫球蛋白等治疗后好转14例,在院死亡19例,病情加重放弃治疗出院12例。结论 HPS病因复杂,临床表现多样,病情凶险、病死率高,积极针对原发病治疗及糖皮质激素、免疫抑制剂、人免疫球蛋白治疗可改善预后。

关键词: 噬血细胞综合征, 病因, 治疗, 转归

Abstract: Objective To summarize the clinical features of patients with hemophagocytic syndrome (HPS). Methods The underlying diseases, clinical characteristics, laboratory findings, responses to therapy as well as their outcomes of 45 patients with HPS were analyzed retrospectively. Results The underlying diseases included virus infection(n=9), tuberculosis infection(n=2), rheumatic diseases(n=4), malignancies(n=22), unknown origin(n=8). HPS was clinically characterized by high fever(93.3%), hepatomegaly (77.8%), splenomegaly(80%), lymphadenopathy(55.6%), skin rash(24.4%), gastrointestinal hemorrhage(22.2%), renal (35.6%) and central nervous systern involvement(15.6%), five patients presented with disseminated intravascular coagulation(DIC)(11.1%). Laboratory data mainly manifested with cytopenia(100%), liver dysfunction(77.8%), hypofibrinogenemia(62.8%), hypertriglyceridemia (71.1%) , serum ferritin>500μg/L(87.5%), low NK-cell activity(92.9%) and hemophagocytosis in bone marrow(100%). Based on treating underlying diseases and use of corticosteroids and immunosuppressive agents in combination with intravenous immunoglobulins(IVIG) therapy, 14 patients recovered, 19 patients died in the hospital, and other 12 cases give up treatment because of exacerbation of the disease. Conclusion There are various underlying diseases and clinical manifestations for HPS. HPS is always extremely dangerous situation with high mortality. Underlying diseases treatments, corticosteroids, immunodepressant and IVIG may improve the prognosis of HPS.

Key words: hemophagocytic syndrome, underlying diseases, treatment, prognosis