噬血细胞综合征诊治研究进展
Research Progress on Diagnosis and Treatment of Hemophagocytic Syndrome
DOI: 10.12677/acm.2025.1541251, PDF,   
作者: 丁永芳, 刘成军：重庆医科大学附属儿童医院重症医学科，儿童发育疾病研究教育部重点实验室，国家儿童健康与疾病临床医学研究中心，儿童发育重大疾病国家国际科技合作基地，儿科学重庆市重点实验室，重庆
关键词: 噬血细胞综合征；诊断；治疗；Hemophagocytic Syndrome； Diagnosis； Treatment
摘要: 噬血细胞综合征(HPS)亦被称为噬血细胞性淋巴组织细胞增生症(HLH)，可分为原发性(遗传性) HLH和继发性(获得性) HLH两类，是由多种因素导致的一种严重的危及生命的恶性炎症反应和组织损伤的临床综合征。HLH临床表现复杂多样，症状、体征缺乏特异性，主要表现为发热、肝脾淋巴结肿大、血象异常、凝血障碍等。由于HLH发病机制和临床表现尚未得到充分的认识，容易漏诊、误诊，且病情进展快，病死率很高，因此早期做出诊断并尽早进行相应的治疗非常关键。本文针对HLH诊断与治疗的新进展进行系统性综述，旨在为HLH的临床诊疗提供借鉴与参考。
Abstract: Hemophagocytic lymphohistiocytosis (HPS), also known as hemophagocytic lymphohistiocytosis (HLH), can be divided into two types: primary (hereditary) HLH and secondary (acquired) HLH. It is a serious and life-threatening clinical syndrome caused by multiple factors, characterized by malignant inflammatory reactions and tissue damage. The clinical manifestations of HLH are complex and diverse, with symptoms and signs lacking specificity. The main manifestations include fever, enlarged liver and spleen lymph nodes, abnormal blood tests, and coagulation disorders. Due to the insufficient understanding of the pathogenesis and clinical manifestations of HLH, it is easy to miss diagnosis and misdiagnosis, and the disease progresses rapidly with a high mortality rate. Therefore, early diagnosis and corresponding treatment are crucial. This article provides a systematic review of the new developments in the diagnosis and treatment of HLH, aiming to provide reference and guidance for the clinical diagnosis and treatment of HLH.
文章引用：丁永芳, 刘成军. 噬血细胞综合征诊治研究进展[J]. 临床医学进展, 2025, 15(4): 2865-2874. https://doi.org/10.12677/acm.2025.1541251

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