EB病毒感染相关噬血细胞综合征的诊治研究进展

噬血细胞综合征(hemophagocytic syndrome,HS)是一组病因及发病机制复杂.组织病理表现以良性组织细胞增生活化,伴随噬血细胞现象的一类综合征.HS分为原发性和继发性两大类,原发性是指家族遗传相关的免疫缺陷状态,继发性指后天获得所致,其中以EB病毒感染相关最为常见.EB病毒感染相关HS的病情进展快,病死率高,现就该病的诊治进展作一概述.

Progress in diagnosis and treatment of Epstein-Barr virus-associated hemophagocytic syndrome

Hemophagocytic syndrome (HS) is a syndrome characterized with complicated etiology and mechanism,the pathological hallmark of this syndrome is unregulated proliferation and activation of histio-cytes and phagocytosis. HS comprises the primary forms and secondary forms,the primary forms are related to the familial genetic defects,and the secondary forms are caused by acquired infections,especially the Epstein-Barr virus ( EBV) infection. EBV-associated HS is a rapidly progressive disease with a high rate of mortality. In this paper,we aim to review the recent progress in diagnosis and treatment of EBV-associated HS.