Relevance of lymphocyte proliferation to PHA in severe combined immunodeficiency (SCID) and T cell lymphopenia

Roshini S. Abraham, Amrita Basu, Jennifer R. Heimall, Elizabeth Dunn, Alison Yip, Malika Kapadia, Neena Kapoor, Lisa Forbes Satter, Rebecca Buckley, Richard O'Reilly, Geoffrey D.E. Cuvelier, Sharat Chandra, Jeffrey Bednarski, Sonali Chaudhury, Theodore B. Moore, Hilary Haines, Blachy J. Dávila Saldaña, Deepakbabu Chellapandian, Ahmad Rayes, Karin ChenEmi Caywood, Shanmuganathan Chandrakasan, Mark Thomas Vander Lugt, Christen Ebens, Pierre Teira, Evan Shereck, Holly Miller, Victor Aquino, Hesham Eissa, Lolie C. Yu, Alfred Gillio, Lisa Madden, Alan Knutsen, Ami J. Shah, Kenneth DeSantes, Jessie Barnum, Larisa Broglie, Avni Y. Joshi, Gary Kleiner, Jasmeen Dara, Susan Prockop, Caridad Martinez, Talal Mousallem, Joseph Oved, Lauri Burroughs, Rebecca Marsh, Troy R. Torgerson, Jennifer W. Leiding, Sung Yun Pai, Donald B. Kohn, Michael A. Pulsipher, Linda M. Griffith, Luigi D. Notarangelo, Morton J. Cowan, Jennifer Puck, Christopher C. Dvorak, Elie Haddad

Research output: Contribution to journalArticlepeer-review

Abstract

Severe combined immunodeficiency (SCID) is characterized by a severe deficiency in T cell numbers. We analyzed data collected (n = 307) for PHA-based T cell proliferation from the PIDTC SCID protocol 6901, using either a radioactive or flow cytometry method. In comparing the two groups, a smaller number of the patients tested by flow cytometry had <10% of the lower limit of normal proliferation as compared to the radioactive method (p = 0.02). Further, in patients with CD3+ T cell counts between 51 and 300 cells/μL, there was a higher proliferative response with the PHA flow assay compared to the 3H-T assay (p < 0.0001), suggesting that the method of analysis influences the resolution and interpretation of PHA results. Importantly, we observed many SCID patients with profound T cell lymphopenia having normal T cell proliferation when assessed by flow cytometry. We recommend this test be considered only as supportive in the diagnosis of typical SCID.

Original languageEnglish (US)
Article number109942
JournalClinical Immunology
Volume261
DOIs
StatePublished - Apr 2024

All Science Journal Classification (ASJC) codes

  • Immunology and Allergy
  • Immunology

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