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http://dx.doi.org/10.1016/j.chstcc.2024.100098 | DOI Listing |
Am J Hematol
September 2025
Department of Hematology, The First Affiliated Hospital of Nanjing Medical University, Jiangsu Province Hospital, Nanjing, China.
Lymphoma-associated hemophagocytic lymphohistiocytosis (LA-HLH) is a life-threatening hyperinflammatory syndrome, and hierarchical management based on a prognostic model is important. The endothelial activation and stress index (EASIX) score has demonstrated prognostic utility in recipients of allogeneic stem cell transplantation and chimeric antigen receptor (CAR) T-cell therapy. However, its role in LA-HLH remains unestablished.
View Article and Find Full Text PDFThe leukocyte NADPH oxidase 2 (NOX2) is an important regulator of inflammatory responses, independent of its antimicrobial activity. Inactivating mutations in NOX2 cause chronic granulomatous disease (CGD), a severe immunodeficiency associated with recurrent infections and dysregulated neutrophilic inflammation. Recurrent oral ulcers, stomatitis, gingivitis, and other inflammatory issues affecting the oral mucosa have been observed in patients with CGD; however, the underlying mechanisms are not known.
View Article and Find Full Text PDFFront Immunol
September 2025
Beijing Tsinghua Changgung Hospital, Department of Gastroenterology, Beijing, China.
Introduction: Hemophagocytic lymphohistiocytosis (HLH) is a rare but life-threatening hyperinflammatory syndrome. Patients with inflammatory bowel diseases (IBD) appear to be at increased risk of developing HLH, potentially due to immunosuppressive therapies. However, the epidemiology, clinical characteristics, management strategies, and outcomes of HLH in this population remain poorly understood.
View Article and Find Full Text PDFFront Immunol
September 2025
Hematology and Bone Marrow Transplantation Unit, IRCCS San Raffaele Scientific Institute, Milan, Italy.
Hemophagocytic lymphohistiocytosis (HLH) is a hyper-inflammatory syndrome characterized by deficient NK-cell activity, cytokine storm and altered T-cell immunity, potentially sustained by multiple triggers. Recently, a hyperinflammatory condition resembling HLH has emerged as a potential complication of CAR T-cells. HLH represents a diagnostic conundrum due to its rarity, non-specific presentation and lack of validated biomarkers.
View Article and Find Full Text PDFInt J Biol Macromol
August 2025
Gene Therapy Laboratory, Department of Integrative Biology, School of Bio Sciences and Technology, Vellore Institute of Technology, Vellore 632 014, TN, India. Electronic address:
Cytotoxic lymphocyte pore-forming protein termed perforin-1 (PFP) produced by NK and CD8 T cells plays a critical role in viral and tumor-related immunity. Deleterious mutations in the gene encoding PFP lead to its dysfunction and impaired cytotoxic activity, causing hemophagocytic lymphohistiocytosis. Familial hemophagocytic lymphohistiocytosis type 2 is a rare, life-threatening, hyperinflammatory disorder that predominantly affects infants and children.
View Article and Find Full Text PDF