Niu Yu, He Ai-Li, Zhang Wang-Gang
Department of Hematology, The Second Hospital, Xi'an Jiaotong University, Xi'an 710004, Shaanxi Province, China.
Zhongguo Shi Yan Xue Ye Xue Za Zhi. 2010 Feb;18(1):262-7.
Hemophagocytic lymphohistiocytosis (HLH) is named as hemophagocytic syndrome (HPS) and is a complicated disease with reactive hyperplasia of mononuclear/macrophagocytic system. This disease characterised by release of massive cytokines and severe functional destruction of visceral organs, which results from immune function disturbance causing by various pathogenic factors. The cardinal clinical symptoms of HLH are prolonged fever, hepatosplenomegaly, cytopenia, elevated ferritin and triglycerides, low fibrinogen, symptom in nerve system and so on. Nevertheless, impaired function of natural killer cells and cytotoxic T-cell is characteristic for HLH. HLH has of two different types that may be difficult to distinguish from one another: a primary and a secondary form. The combined immunochemotherapy of dexamethasone, etoposide and cyclosporin A and hematopoietic stem cell transplantation are considered as the effective therapies for HLH. In this article, the recent advance in research on the etiological factors, pathogenesis, clinical manifestations, laboratory examination, diagnosis as well as recommended therapy of HLH were reviewed.
噬血细胞性淋巴组织细胞增生症（HLH）又称为噬血细胞综合征（HPS），是一种单核/巨噬细胞系统反应性增生的复杂疾病。该疾病的特征是大量细胞因子释放以及内脏器官严重的功能破坏，这是由各种致病因素引起的免疫功能紊乱导致的。HLH的主要临床症状包括长期发热、肝脾肿大、血细胞减少、铁蛋白和甘油三酯升高、纤维蛋白原降低、神经系统症状等。然而，自然杀伤细胞和细胞毒性T细胞功能受损是HLH的特征。HLH有两种不同类型，可能难以相互区分：原发性和继发性。地塞米松、依托泊苷和环孢素A的联合免疫化疗以及造血干细胞移植被认为是HLH的有效治疗方法。本文综述了HLH在病因、发病机制、临床表现、实验室检查、诊断以及推荐治疗等方面的最新研究进展。
