한빛사논문
- 조회157
Tram T. T. Nguyen 1,4, Yoon Tae Kim 1,4, Geunyeol Jeong 1 and Mirim Jin 1,2,3,*
1Department of Health Sciences and Technology, GAIHST, Gachon University, Incheon, Republic of Korea.
2Lee Gil Ya Cancer and Diabetes Institute, Gachon University, Incheon, Republic of Korea.
3Department of Microbiology, College of Medicine, Gachon University, Incheon, Republic of Korea.
4These authors contributed equally: Tram T. T. Nguyen, Yoon Tae Kim.
*Corresponding author: correspondence to Mirim Jin
Abstract
Secondary hemophagocytic lymphohistiocytosis/macrophage activation syndrome (sHLH/MAS) is a life-threatening immune disorder triggered by rheumatic disease, infections, malignancies, or medications. Characterized by the presence of hemophagocytic macrophages and a fulminant cytokine storm, sHLH/MAS leads to hyperferritinemia and multiorgan failure and rapidly progresses to death. The high mortality rate and the lack of specific treatments necessitate the development of a new drug. However, the complex and largely unknown immunopathologic mechanisms of sHLH/MAS, which involve dysfunction of various immune cells, diverse etiologies, and different clinical contexts make this effort challenging. This review introduces the terminology, diagnosis, and clinical features of sHLH/MAS. From a translational perspective, this review focuses on the immunopathological mechanisms linked to various etiologies, emphasizing potential drug targets, including key molecules and signaling pathways. We also discuss immunomodulatory biologics, existing drugs under clinical evaluation, and novel therapies in clinical trials. This systematic review aims to provide insights and highlight opportunities for the development of novel sHLH/MAS therapeutics.
논문정보
- Review Paper
- 2024년 03월 (BRIC 등록일 2024-03-11)
- 국내 연구진
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