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摘要: 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。Abstract: 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.
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Key words:
- malignancy /
- hemophagocytic lymphohistiocytosis (HLH) /
- diagnosis /
- treatment
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