成人嗜血细胞综合征.pdfVIP

2015 NO.13 中外医疗 ChinaForeign MedicalTreatment 成人嗜血细胞综合征 朱 俪袁孟海涛 浙江大学医学院附属第一医院血液科袁浙江杭州 310003 [摘要] 嗜血细胞综合征渊HPS冤是一类危及生命的高炎症反应综合症袁并非一种独立的疾病遥HPS分为原发性和继发性袁原发 性多见于儿童袁继发性可由多种因素诱发袁包括感染尧肿瘤尧自身免疫性疾病等遥目前对HPS的报道以儿童居多袁但成人HPS 的发生率与日俱增遥对于嗜血细胞综合征缺乏认识可能导致误诊及病人预后差袁早期诊断对于提高患者存活率是十分必要 的遥 该文旨在对嗜血细胞综合的病因尧诊断尧治疗等的最新进展做一综述遥 [关键词] 嗜血细胞综合征曰成人曰诊断曰治疗 [中图分类号] R55 [文献标识码] A [文章编号] 1674-074圆渊圆园员5冤05渊a冤原园191-03 Adult Haemophagocytic Synrome ZHULi袁MENG Hai-tao Department of Hematology, The First Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou袁Zhejiang Province袁 310003 China [Abstract]Haemophagocytic syndrome(HPS)is a kind of life-threatening hyperinflammatory response syndrome and not indepen鄄 dent disease.HPS is divided into primary and secondary,primary is more common in children,and secondary can be induced by a variety of factors,including infection,tumor,autoimmune disease and so on.At present ,the reports of HPS in children are majority, butthe incidenceofadult HPSgroweachpassing day.Lackofawarenessof HPSmayleadto misdiagnosisandpoor prognosis,early diagnosisisvery importantto mprove the life quality andthe survivalrate of people.This article aims to review the recent progress ofthe pathogeny,diagnosis,treatment andsoonofHPS. [Keywords] Haemophagocytic syndrome曰Adult曰Diagnosis曰Treatment [2] 噬血细胞综合症渊hemophagocytic syndrome袁HPS冤亦称噬血 平均年龄50岁 遥 细胞性淋巴组织细胞增生症(HLH)袁又称噬血细胞性网状细胞增 2 病因