摘要
噬血细胞综合征(HPS)不是一种独立的疾病,而是一组临床综合征,临床特征是持续性发热、肝脾大、血细胞减少及在骨髓、肝脾和淋巴结的组织细胞噬血现象。HPS分为原发性和继发性两种类型。恶性肿瘤特别是恶性淋巴瘤是引起成人继发性HPS的主要原因。结外NK/T细胞淋巴瘤相关性HPS(NK/T-LAHS)临床上少见且致死率高,其临床表现不典型,早期诊断困难,治疗效果差,预后不佳,值得临床医师重视。
Hemophagocytic syndrome（HPS） is not an independent disease but a clinical syndrome with a group of clinical features such as persistent fever,hepatosplenomegaly,cytopenia,and hemophagocytosis in bone marrow,liver,spleen,and lymphonode.HPS can be classified into primary and secondary types.Malignancies,particularly malignant lymphoma,are the leading causes of HPS in adults.Extranodal NK/T-cell lymphoma-associated HPS（NK/T-LAHS） is a rare but life-threatening desease.Due to atypical clinical manifestations,difficulties in early diagnosis,poor efficacy and poor prognosis,special attention to NK/TLAHS should be paid by the clinicians.
出处
《医学综述》
2015年第7期1200-1203,共4页
Medical Recapitulate
