噬血细胞综合征2例临床报告及文献复习

Two clinical reports and literature review of hemophagocytic syndrome

噬血细胞综合征,又称噬血细胞性淋巴组织细胞增生症,是一组因遗传性或获得性免疫缺陷导致以过度炎症反应为特征的疾病。其发生机制可能是致病因素激活T淋巴细胞促使其分泌大量细胞因子,引起T细胞介导的细胞因子风暴,从而刺激和激活大量组织细胞增生并吞噬细胞,引起高热、肝脾淋巴结肿大、黄疸和肝功能异常、凝血障碍和全血细胞减少为表现的凶险的综合病征。介绍噬血细胞综合征病例2例并结合文献分析其诊疗思路。

Hemophagocytic lymphohistiocytosis（HLH）, also known as hemophagocytic syndrome, is an uncommon hematologic disorder.It is a life-threatening disease of severe hyperinflammation caused by uncontrolled proliferation of activated lymphocytes and macrophages, characterized by proliferation of morphologically benign lymphocytes and macrophages that secrete high amounts of inflammatory cytokines. It is classified as one of the cytokine storm syndromes. In this article, two clinical cases of our department were reported. Combined with literature review, it was analyzed the thinking way of diagnosing and treating.