淋巴瘤相关噬血细胞综合征的诊疗进展

Progress in Diagnosis and Treatment of Lymphoma-Associated Hemophagocytic Syndrome

噬血细胞综合征(Hemophagocytic Syndrome, HPS)又称噬血细胞性淋巴组织细胞增多症(Hemphagocytic Lymphohistiocytosis, HLH)是一种由于原发性或继发性免疫异常导致的一类罕见但致命的过度炎症反应综合征,主要症状包括长期发热、血细胞减少、肝脾肿大。淋巴瘤相关噬血细胞综合征(Lymphoma Associated Hemophagocytic Syndrome, LAHS)患者多数病情危重、误诊率、延诊率和死亡率较高,本文就LAHS诊断及治疗等方面进行综述,旨在提高对LAHS的认识,指导临床治疗。

Hemophagocytic syndrome (HPS), also known as hemophagocytic lymphohistiocytosis (HLH), is a rare but fatal excessive inflammatory response syndrome caused by primary or secondary immune abnormalities. The main symptoms include long-term fever, cytopenia and hepatosplenomegaly. Most patients with lymphoma-associated hemophagocytic syndrome (LAHS) are in critical condition, with high misdiagnosis rate, delayed diagnosis rate and mortality rate, this article reviews the diagnosis and treatment of LAHS in order to improve the understanding of LAHS and guide clinical treatment.