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Journal Article Review Research Support, Non-U.S. Gov't Epublish 6 citations

PubMed: 39511607 DOI: 10.1186/s13045-024-01621-x

Hemophagocytic lymphohistiocytosis: current treatment advances, emerging targeted therapy and underlying mechanisms.

J Hematol Oncol 17(1):106

Authors: Yijun Wu, Xu Sun, Kai Kang, Yuqi Yang, He Li, Ailin Zhao, Ting Niu

Affiliations

  • Department of Hematology, West China Hospital, Sichuan University, Chengdu, Sichuan, China. Division of Thoracic Tumor Multimodality Treatment, Cancer Center, West China Hospital, Sichuan University, Chengdu, Sichuan, China. State Key Laboratory of Biotherapy, Collaborative Innovation Center of Biotherapy, West China Hospital, Sichuan University, Chengdu, Sichuan, China. National Facility for Translational Medicine (Sichuan), West China Hospital, Sichuan University, Chengdu, Sichuan, China.
  • Department of Hematology, West China Hospital, Sichuan University, Chengdu, Sichuan, China. State Key Laboratory of Biotherapy, Collaborative Innovation Center of Biotherapy, West China Hospital, Sichuan University, Chengdu, Sichuan, China. National Facility for Translational Medicine (Sichuan), West China Hospital, Sichuan University, Chengdu, Sichuan, China.
  • Division of Thoracic Tumor Multimodality Treatment, Cancer Center, West China Hospital, Sichuan University, Chengdu, Sichuan, China. State Key Laboratory of Biotherapy, Collaborative Innovation Center of Biotherapy, West China Hospital, Sichuan University, Chengdu, Sichuan, China. National Facility for Translational Medicine (Sichuan), West China Hospital, Sichuan University, Chengdu, Sichuan, China.
  • West China School of Medicine, Sichuan University, Chengdu, Sichuan, China.
  • Department of Hematology, West China Hospital, Sichuan University, Chengdu, Sichuan, China.
  • State Key Laboratory of Biotherapy, Collaborative Innovation Center of Biotherapy, West China Hospital, Sichuan University, Chengdu, Sichuan, China.
  • National Facility for Translational Medicine (Sichuan), West China Hospital, Sichuan University, Chengdu, Sichuan, China.
  • Department of Hematology, West China Hospital, Sichuan University, Chengdu, Sichuan, China. State Key Laboratory of Biotherapy, Collaborative Innovation Center of Biotherapy, West China Hospital, Sichuan University, Chengdu, Sichuan, China. National Facility for Translational Medicine (Sichuan), West China Hospital, Sichuan University, Chengdu, Sichuan, China.
  • Department of Hematology, West China Hospital, Sichuan University, Chengdu, Sichuan, China. State Key Laboratory of Biotherapy, Collaborative Innovation Center of Biotherapy, West China Hospital, Sichuan University, Chengdu, Sichuan, China. National Facility for Translational Medicine (Sichuan), West China Hospital, Sichuan University, Chengdu, Sichuan, China.

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rapidly progressing, life-threatening syndrome characterized by excessive immune activation, often presenting as a complex cytokine storm. This hyperactive immune response can lead to multi-organ failure and systemic damage, resulting in an extremely short survival period if left untreated. Over the past decades, although HLH has garnered increasing attention from researchers, there have been few advancements in its treatment. The cytokine storm plays a crucial role in the treatment of HLH. Investigating the detailed mechanisms behind cytokine storms offers insights into targeted therapeutic approaches, potentially aiding in early intervention and improving the clinical outcome of HLH patients. To date, there is only one targeted therapy, emapalumab targeting interferon-γ, that has gained approval for primary HLH. This review aims to summarize the current treatment advances, emerging targeted therapeutics and underlying mechanisms of HLH, highlighting its newly discovered targets potentially involved in cytokine storms, which are expected to drive the development of novel treatments and offer fresh perspectives for future studies. Besides, multi-targeted combination therapy may be essential for disease control, but further trials are required to determine the optimal treatment mode for HLH.

MeSH

Cytokines, Hematology, Multiple Chronic Conditions, Cytokine Release Syndrome, Therapeutics, Lymphohistiocytosis, Hemophagocytic

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