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Journal Article Ppublish 2 citations

PubMed: 39226525 DOI: 10.1002/eji.202451140

CD38/HLA-DRCD8 T lymphocytes display pathogen-specific expansion regardless of hemophagocytic lymphohistiocytosis.

Eur J Immunol 54(11):e2451140

Authors: Lorenzo Lodi, Walter Maria Sarli, Silvia Ricci, Laura Pisano, Silvia Boscia, Maria Vincenza Mastrolia, Sara Malinconi, Eleonora Fusco, Elena Sieni, Giuseppe Indolfi, Gabriele Simonini, Luisa Galli, Chiara Azzari

Affiliations

  • Department of Health Sciences, University of Florence, Florence, Italy.
  • Immunology Unit, Meyer Children's Hospital IRCCS, Florence, Italy.
  • Rheumatology Unit, Meyer Children's Hospital IRCCS, Florence, Italy.
  • Pediatric Hematology-Oncology Department, Meyer Children's Hospital IRCCS, Florence, Italy.
  • Hepatology Unit, Meyer Children's Hospital IRCCS, Florence, Italy.
  • Department NEUROFARBA, University of Florence, Florence, Italy.
  • Infectious Disease Unit, Meyer Children's Hospital IRCCS, Florence, Italy.

Abstract

The characteristic expansion of T CD38/HLA-DRCD8 lymphocytes observed in hemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS) proved able to distinguish HLH/MAS from sepsis and systemic juvenile idiopathic arthritis. However, the performance of this marker in differentiating HLH/MAS from other pediatric febrile conditions with similar clinical onset and yet entirely different treatments remains unexplored. CD38/HLA-DRCD8 frequencies measured in the peripheral fresh blood of pediatric patients attended for suspicion of HLH/MAS were retrospectively recorded and clinical characteristics were retrieved. CD38/HLA-DRCD8 frequencies in HLH/MAS patients (15 patients; median: 22.0%, IQR: 11.0-49.0%) were compared with those who presented febrile conditions other-than-HLH (28 patients; median: 13.0%, IQR: 3.9-28.7%; p = 0.24). HLH and non-HLH patients were subsequently regrouped based on the presence of an identified infection (22 patients; median: 27.0%, IQR: 15.2-72.1%) and compared with those without infections (21 patients; median: 7.6%, IQR: 3.7-24.3%; p = 0.0035). CD38/HLA-DRCD8 percentages were significantly higher only in the infection group compared with the noninfection one, with a patent pathogen-specific expansion in Epstein-Barr virus primoinfection and visceral leishmaniasis regardless of the presence of HLH. CD38/HLA-DRCD8 frequencies do not appear as an HLH-specific marker as they naturally expand in other clinical situations that are common in childhood and may mimic HLH initial presentation.

MeSH

Lymphocytes, Allergy And Immunology, T-Lymphocytes, Lymphohistiocytosis, Hemophagocytic, Infections

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