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噬血细胞综合征（HPS）是一种罕见的良性单核巨噬细胞系统反应增生性疾病。临床以高热、肝脾肿大、全血细胞减少和凝血障碍为主要表现。未及时诊断和治疗病死率很高。其病因分为原发性和继发性两大类,前者主要与遗传有关,后者与基础疾病密切相关。HPS的发病机制仍未明确,但多认为免疫调节系统异常起主要作用。目前,对于原发性HPS行骨髓移植是根本性治疗,而对于继发性HPS的治疗应该基础疾病与HPS并重,否则预后很差。病毒相关性HPS,尤其是EB病毒相关性 HPS的患者病死率相对较高。
Hemophagocytic syndrome (HPS) is a rare, systemic, proliferative disease of the monocytic macrophage. Clinical fever, hepatosplenomegaly, pancytopenia and coagulation disorders as the main performance. Not timely diagnosis and treatment of high mortality. The etiology is divided into two major categories of primary and secondary, the former mainly with the genetic, which is closely related with underlying diseases. The pathogenesis of HPS is still not clear, but many believe that the abnormal immune system plays a major role. Currently, bone marrow transplantation for primary HPS is a fundamental treatment, and for secondary HPS treatment should be based on the same disease and HPS, otherwise the prognosis is poor. Patients with virus-related HPS, especially EB virus-associated HPS, have a relatively high case fatality rate.