Zoref-Lorenz Adi, Rocco Joseph, Schwartz Daniella M, Jordan Michael
Hematology Institute, Department of Medicine, Meir Medical Center, Tchernichovsky Street 59, Kfar Saba 4428164, Israel; Faculty of Medicine and Health Sciences, Tel Aviv University, Tel Aviv, Israel.
Laboratory of Immunoregulation, National Institute of Allergy and Infectious Diseases, National Institutes of Health, NIH Clinical Center Building 10, Room 11B-17 10 Center Drive, Bethesda, MD 20892, USA. Electronic address: https://twitter.com/JMRocco5.
Hematol Oncol Clin North Am. 2025 Jun;39(3):577-596. doi: 10.1016/j.hoc.2025.02.007. Epub 2025 Apr 12.
Secondary hemophagocytic lymphohistiocytosis (sHLH) is a life-threatening hyperinflammatory syndrome triggered by infections, malignancies, or rheumatologic conditions. Effective management requires identifying and treating the acute trigger while addressing underlying factors and calming the inflammatory response. Like sepsis, sHLH represents a cytokine storm resulting from diverse triggering events rather than a standalone diagnosis. This review synthesizes current literature and the authors' clinical experience to provide a comprehensive framework for diagnosing and managing sHLH, emphasizing the importance of tailored, trigger-specific interventions. Emerging diagnostic tools and therapeutic strategies and improved mechanistic understanding of sHLH hold promise for improving outcomes in this challenging condition.
继发性噬血细胞性淋巴组织细胞增生症（sHLH）是一种由感染、恶性肿瘤或风湿性疾病引发的危及生命的高炎症综合征。有效的管理需要识别并治疗急性触发因素，同时解决潜在因素并平息炎症反应。与脓毒症一样，sHLH代表由多种触发事件导致的细胞因子风暴，而非一个独立的诊断。本综述综合了当前文献和作者的临床经验，以提供一个用于诊断和管理sHLH的全面框架，强调针对特定触发因素进行个性化干预的重要性。新兴的诊断工具和治疗策略以及对sHLH机制理解的改善，有望改善这种具有挑战性疾病的治疗结果。
