嗜血细胞性淋巴组织细胞增生症的发病机制、诊断及治疗进展

Review on the progress of pathogenesis,diagnosis and treatment of hemophagocytic lymphohistiocytosis

嗜血细胞性淋巴组织细胞增生症（hemophago-cytic lymphohistiocytosis,HLH）,又称嗜血细胞综合征（Hemophagocytic Syndrome,HPS）,是以过度炎症反应综合征为共同临床表现的一组疾病,由于机体免疫功能紊乱导致活化的淋巴细胞及组织细胞过度增生、大量细胞因子释放,进而引起多脏器严重受损;临床表现包括发热、肝脾肿大、血细胞减少等,同时伴有高甘油三脂血症、低纤维蛋白原血症、肝功能异常等。组织病理学检查可见大量的淋巴细胞,成熟的巨噬细胞、组织细胞浸润骨髓或者其他脏器。

In order to provide some evidence for the diagnosis and treatment of hemophagocytic lymphohistiocytosis（HLH）,we reviewed the progress on pathogenesis,diagnosis and treatment of HLH.We consulted domestic and foreign literatures about HLH,and analyzed systematically.Due to complex pathogenesis,wide variety of clinical manifestations,rapid progression and lackness of specific diagnosis index,early diagnosis of HLH is difficult and the prognosis is poor.Along with the study progress on the pathogenesis and the application of HLH-2004in the treatment of HLH,the diagnosis,treatment and prognosis will be gradually improved.Recognization of the progress on pathogenesis,diagnosis and treatment of HLH is important for clinicians.