恶性肿瘤相关噬血综合征的研究进展

Research progress on malignancy-associated hemophagocytic lymphohistiocytosis

噬血综合征(hemophagocytic lymphohistiocytosis,HLH)是一种少见的疾病,由于机体分泌大量细胞因子产生过度炎症反应,进而导致多器官、多系统受累的临床综合征。恶性肿瘤相关噬血综合征(malignancy-associated hemophagocytic lymphohistiocytosis,MA-HLH)患者多数病情危重、误诊率、延诊率和死亡率较高,对其认识不断被重视和深化。尤其是在免疫检测点抑制剂的广泛应用之后,MA-HLH的发生越来越多。本文结合近年来这一领域相关研究进展,从流行病学、易患因素、病理生理、诊断标准及治疗等方面进行综述。

Hemophagocytic lymphohistiocytosis(HLH)is a life-threatening disease characterized by uncontrolled cytotoxic T cells and macrophages.Contributing causes include heredity,infection,autoimmune disease,and tumors,which stimulate the activation of macrophages and secretion of a large number of inflammatory factors.Most malignancy-associated hemophagocytic lymphohistiocytosis(MA-HLH)patients are in critical condition at time of diagnosis,due a high rate of delayed diagnosis and misdiagnosis,resulting in highmortality.With extensive application of immune checkpoint inhibitors,incidence of MA-HLH is increasing.This article reviews recent research progress in this field,focused on epidemiology,predisposing factors,pathophysiology,diagnostic criteria,and treatment.