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Advances in diagnosis and treatment of adult hemophagocytic syndrome
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Yan-Quan Liu1, Xiao-Jun Chen2, Min-Juan Zeng3, Jian-Zhen Shen4, *
Medical Journal of Chinese People’s Liberation Army | 2024, 49(8) : 952 - 958
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Medical Journal of Chinese People’s Liberation Army | 2024, 49(8): 952-958
• Review •
Advances in diagnosis and treatment of adult hemophagocytic syndrome
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Yan-Quan Liu1, Xiao-Jun Chen2, Min-Juan Zeng3, Jian-Zhen Shen4, *
Affiliations
  • 1Department of Hematology, the First School of Clinical Medicine, Guangdong Medical University, Dongguan, Guangdong 523808, China
  • 2Department of Hematology, the Affiliated Hospital of Putian University, Putian, Fujian 351100, China
  • 3Department of Anatomy, School of Basic Medicine, Guangdong Medical University, Dongguan, Guangdong 523808, China
  • 4Department of Hematology, Fujian Medical University Union Hospital, Fuzhou, Fujian 350001, China
Published: 2024-08-28 doi: 10.11855/j.issn.0577-7402.0363.2024.0625
Outline
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Hemophagocytic syndrome (HPS), also known as hemophagocytic lymphohistiocytosis (HLH), is a rare and highly malignant hematologic disease with a poor prognosis. It can be divided into two categories: primary HLH and secondary HLH. HLH is characterized by a large number of abnormal immune cells that continuously activate and regulate out of control, leading to systemic inflammatory factor storm and multiple organ failure. Clinical manifestations mainly include persistent malignant hyperthermia, pancytopenia, hepatosplenomegaly, and hemophagocytosis in tissues and organs. The pathogenesis of adult HLH is complex, with rapid onset and rapid disease progression, and the fatality rate remains high. The main causes of death in HLH patients are multiple organ failure, abnormal coagulation and septic shock. Due to the specificity of HLH and the lack of effective understanding of the severity and prognosis in clinical practice, some HLH patients are misdiagnosed or overlooked, missing the best opportunity for diagnosis and treatment. Therefore, this review systematically explores and discusses the latest diagnostic and treatment progress of adult HLH, aiming to provide reference for clinical diagnosis, treatment and prognosis assessment of HLH.

hemophagocytic syndrome  /  malignant hematological diseases  /  diagnosis and differentiation  /  treatment  /  prognosis
Yan-Quan Liu, Xiao-Jun Chen, Min-Juan Zeng, Jian-Zhen Shen. Advances in diagnosis and treatment of adult hemophagocytic syndrome[J]. Medical Journal of Chinese People’s Liberation Army, 2024 , 49 (8) : 952 -958 . DOI: 10.11855/j.issn.0577-7402.0363.2024.0625
Funding
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  • National Clinical Key Specialty Construction Project(Min Medical Administration 2021-76)
  • Project of Fujian Clinical Medical Research Center for Hematologic Malignancies(2020Y2006)
Appendix
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Year 2024 volume 49 Issue 8
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Article Info
doi: 10.11855/j.issn.0577-7402.0363.2024.0625
  • Receive Date：2024-03-21
  • Online Date：2025-11-21
  • Published：2024-08-28
Article Data
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History
  • Received：2024-03-21
  • Accepted：2024-04-25
Funding
National Clinical Key Specialty Construction Project(Min Medical Administration 2021-76)
Project of Fujian Clinical Medical Research Center for Hematologic Malignancies(2020Y2006)
Affiliations
    1Department of Hematology, the First School of Clinical Medicine, Guangdong Medical University, Dongguan, Guangdong 523808, China
    2Department of Hematology, the Affiliated Hospital of Putian University, Putian, Fujian 351100, China
    3Department of Anatomy, School of Basic Medicine, Guangdong Medical University, Dongguan, Guangdong 523808, China
    4Department of Hematology, Fujian Medical University Union Hospital, Fuzhou, Fujian 350001, China

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