摘要
目的 探讨噬血细胞综合征（HPS）的临床特征与预后的关系.方法 对2011年1月至2014年6月确诊的102例HPS患者的病因、临床表现、实验室检查及治疗进行回顾性分析.结果 102例患者中,肿瘤相关性HPS最多见,共42例（41.18％）.噬血细胞综合征2004诊断标准中各指标符合率分别为：发热100％（102例）,脾大79.41％（81例）,两系或三系血细胞减少81.37％（83例）,高三酰甘油血症38.24％（39例）,低纤维蛋白原血症53.92％（55例）,铁蛋白升高99.02％（101例）,骨髓噬血现象76.60％（72例）.用Cox单因素分析显示,患者年龄、性别、血小板计数、清蛋白水平与预后有关,差异有统计学意义（P＜0.05）.多因素生存分析显示,患者年龄、血小板计数、清蛋白水平与预后有关,差异有统计学意义（P＜0.05）.结论 HPS可由多种病因所致,肿瘤相关性多见,临床表现多样,发病年龄、血小板计数、清蛋白水平是影响预后的独立危险因素.
Objective To explore the relationship between clinical features and prognosis in adult hemophagocytic syndrome （HPS）.Methods A retrospective study was carried out in 102 adult patients with HPS from January 2011 to June 2014.Results Of the 102 cases,most （41.18 %,42/102） cases were secondary cancer.According to 2004 criteria,the incidence rate of fever was 100 % （102/102）,splenomegaly 79.41% （81/102）,cytopenias 81.37 % （83/102）,hypertriglyceridemia 38.24 % （39/102）,hypofibrinogenemia 53.92 % （55/102）,elevated ferrtin 99.02 % （101/102）,hemophagocytosis in bone marrow 76.60 % （72/102）.Univariate analysis with Cox showed that age,sex,platelet counts and albumin were associated with the prognosis,and the differences were statistically significant （P 〈 0.05）.Analyzed by multivariate survival analysis,age,platelet counts and albumin were associated with the prognosis,and the differences were statistically significant （P 〈 0.05）.Conclusions HPS is caused by a variety of etiological factors,and the most common cause is cancer,with diverse clinical features.Age,platelet counts and albumin are the independent risk factors of prognosis.
出处
《白血病．淋巴瘤》
CAS
2015年第5期295-297,301,共4页
Journal of Leukemia & Lymphoma
关键词
噬血细胞淋巴组织细胞增生症
临床特征
实验室检查
预后
Hemophagocytic lymphohistiocytosis
Clinical features
Laboratory characteristic
Prognosis
