Bibliographic Details
| Title: |
T-ing up the storm: pathogenic cycling lymphocytes in the biology of macrophage activation syndrome. |
| Authors: |
Lam, Michael T.1 (AUTHOR), Jiang, Connie L.1,2 (AUTHOR), Lee, Pui Y.1 (AUTHOR) pui.lee@childrens.harvard.edu |
| Source: |
Pediatric Rheumatology. 3/17/2025, Vol. 23 Issue 1, p1-10. 10p. |
| Subject Terms: |
*MACROPHAGE activation syndrome, *CYTOKINE release syndrome, *T cells, *MYELOID cells, *HEMOPHAGOCYTIC lymphohistiocytosis |
| Abstract: |
Background: Hemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS) are potentially fatal cytokine storm syndromes with clinical features including fever, pancytopenia, hepatosplenomegaly, coagulopathy, and progressive multiorgan system dysfunction. Mechanistically, HLH / MAS are driven by persistent activation of lymphoid and myeloid cells, but our understanding of the pathogenic cell populations remains incomplete. Main body: In this Perspectives article, we provide an overview of the biology of HLH / MAS and the critical role of interferon-g in disease pathogenesis. We discuss the recent discovery of cycling lymphocytes in HLH / MAS marked by expression of CD38 and HLA-DR, which are primary producers of IFN-γ. The expansion of cycling lymphocytes correlates with disease activity and helps to distinguish HLH / MAS from clinical mimics. We demonstrate an approach to quantify CD38+HLA-DR+ cycling lymphocytes and evaluate their utility as a diagnostic biomarker for HLH / MAS. Lastly, we discuss the treatment of MAS, including potential therapeutic options to target these pathogenic lymphocytes. Conclusion: Understanding of biology of cycling lymphocytes in HLH / MAS will facilitate the development of novel therapeutic approaches to overcome these fatal hyperinflammatory disorders. [ABSTRACT FROM AUTHOR] |
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