Roxane Labrosse, Ines Boufaied, Benoîte Bourdin, Saideep Gona, Haley E Randolph, Brent R Logan, Sara Bourbonnais, Chloé Berthe, Wendy Chan, Rebecca H Buckley, Roberta E Parrott, Geoffrey D E Cuvelier, Neena Kapoor, Sharat Chandra, Blachy J Dávila Saldaña, Hesham Eissa, Fred D Goldman, Jennifer Heimall, Richard O'Reilly, Sonali Chaudhury, Edward A Kolb, Shalini Shenoy, Linda M Griffith, Michael Pulsipher, Donald B Kohn, Luigi D Notarangelo, Sung-Yun Pai, Morton J Cowan, Christopher C Dvorak, Élie Haddad, Jennifer M Puck, Luis B Barreiro, Hélène Decaluwe
BACKGROUND: Severe combined immunodeficiency (SCID) comprises rare inherited disorders of immunity that require definitive treatment through hematopoietic cell transplantation (HCT) or gene therapy for survival. Despite successes of allogeneic HCT, many SCID patients experience incomplete immune reconstitution, persistent T-cell lymphopenia, and poor long-term outcomes. OBJECTIVE: We hypothesized that CD4+ T-cell lymphopenia could be associated with a state of T-cell exhaustion in previously transplanted SCID patients...
August 17, 2022: Journal of Allergy and Clinical Immunology