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The role of C-reactive protein and ferritin in the diagnosis of HLH, adult-onset still's disease, and COVID-19 cytokine storm.
Sci Rep
December 2024
International Collaboration On Repair Discoveries, School of Biomedical Engineering, University of British Columbia, Vancouver, BC, Canada.
Cytokine storm syndromes such as hemophagocytic lymphohistiocytosis (HLH), Adult-onset Still's disease (AOSD), and COVID-19 cytokine storm (CCS) are characterized by markedly elevated inflammatory cytokines. However clinical measurement of serum cytokines is not widely available. This study examined the clinical utility of C-reactive protein (CRP) and ferritin, two inexpensive and widely available inflammatory markers, for distinguishing HLH from AOSD and CCS.
View Article and Find Full Text PDFInvestigating p.Ala1035Val in NPC1: New Cellular Models for Niemann-Pick Type C Disease.
Int J Mol Sci
November 2024
Research and Development Unit, Department of Human Genetics, National Institute of Health Doutor Ricardo Jorge (INSA, I. P.), Rua Alexandre Herculano 321, 4000-055 Porto, Portugal.
Article Synopsis
- * The p.Ala1035Val variant is the most common in Portugal and is linked to a potential SNP (p.Ile858Val) that may affect disease progression.
- * Research using in vitro models reveals that the p.Ala1035Val variant, especially when combined with p.Ile858Val, shows decreased lysosomal trafficking similar to p.Ile1061Thr, suggesting the need for new therapeutic approaches.
[Clinical Crrelation and Prognostic Analysis of ALBI Score in Secondary Hemophagocytic Syndrome in Children].
Zhongguo Shi Yan Xue Ye Xue Za Zhi
October 2024
Department of Pediatrics, The Affiliated Hospital of Zunyi Medical University； Department of Pediatrics, Guizhou Children's Hospital, Collaborative Innovation Center for Tissue Injury Repair and Regenerative Medicine of Zunyi Medical University, Zunyi 563000，Guizhou Province, China.
Objective: To explore the clinical correlation and prognostic value of the Albumin-Bilirubin (ALBI) score in children with secondary hemophagocytic syndrome(sHLH).
Methods: A retrospective analysis was conducted on the data of children's sHLH cases clearly diagnosed in the Affiliated Hospital of Zunyi Medical University from January 2012 to March 2023. Survival analysis was conducted according to the ALBI classification.
Hemophagocytic lymphohistiocytosis associated with immune checkpoint inhibitor use: A review of the current knowledge and future directions.
Blood Cells Mol Dis
February 2025
Division of Hematology/Oncology, Department of Medicine, Beth Israel Deaconess Medical Center, Boston, MA, United States of America. Electronic address:
Hemophagocytic lymphohistiocytosis (HLH) is a severe and often lethal inflammatory syndrome characterized by excessive immune activation leading to fever, cytopenias, and multiorgan involvement. Immune checkpoint inhibitors (ICIs) are central to many contemporary cancer regimens, but their use is associated with immune-related adverse events. Here, we report a case of ICI-induced HLH successfully treated with single agent dexamethasone and provide a scoping review of the literature for cases of ICI-induced HLH with a focus on treatment strategies and outcomes.
View Article and Find Full Text PDFCD68-Negative Histiocytoses with Cardiac Involvement, Associated with COVID-19.
Int J Mol Sci
September 2024
Almazov National Medical Research Centre, 197341 St. Petersburg, Russia.
Histiocytoses are rare diseases characterised by infiltration of affected organs by myeloid cells with a monocyte or dendritic cell phenotype. Symptoms can range from self-resolving localised forms to multisystemic lesions requiring specific treatment. To demonstrate extremely rare cases of CD68-negative cardiac histiocytosis with expression of SARS-CoV-2 antigen in infiltrate cells.
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