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王晶石.噬血细胞综合征的诊疗进展[J].内科急危重症杂志,2025,31(2):108-102
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| DOI：10.11768/nkjwzzzz20250203 |
| 中文关键词: 噬血细胞综合征 诊断 治疗 |
| 英文关键词: |
| 基金项目:国家自然科学基金（82370185；82470183） |
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| 摘要点击次数: 124 |
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| 中文摘要: |
| 摘要 噬血细胞综合征（HLH）是一种遗传性或获得性免疫调节功能异常导致的严重炎症反应综合征。根据病因不同，HLH可以分为原发性HLH和继发性HLH。HLH是一种进展迅速的高致死性疾病，及时诊断和治疗对挽救患者生命至关重要。近年来随着对HLH认知的加深，新药的研发和各种临床实验的开展，HLH已经进入精准诊断和分层治疗时代。本文将结合国内外最新的高质量临床研究和真实世界数据总结原发性和继发性HLH的诊断和治疗进展。 |
| 英文摘要: |
| Abstract Hemophagocytic lymphohistiocytosis (HLH) is a severe inflammatory response syndrome caused by inherited or acquired dysregulation of immune function. Depending on the etiology, HLH can be divided into primary HLH and secondary HLH. HLH is a highly lethal disease that progresses rapidly, and timely diagnosis and treatment are critical to saving the lives of patients. In recent years, with the deepening of the cognition of HLH, the development of new drugs and the development of various clinical trials, HLH has entered the era of accurate diagnosis and stratified treatment. This article will summarize the progress of diagnosis and treatment of primary and secondary HLH by combining the latest high-quality clinical studies and real-world data. |
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