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


Roshini S. Abraham, Nationwide Children’s Hospital
Amrita Basu, Nationwide Children’s Hospital
Jennifer R. Heimall, The Children's Hospital of Philadelphia
Elizabeth Dunn, UCSF School of Medicine
Alison Yip, UCSF School of Medicine
Malika Kapadia, Harvard Medical School
Neena Kapoor, Keck School of Medicine of USC
Lisa Forbes Satter, Baylor College of Medicine
Rebecca Buckley, Duke University Medical Center
Richard O'Reilly, Memorial Sloan-Kettering Cancer Center
Geoffrey D.E. Cuvelier, University of Manitoba
Sharat Chandra, University of Cincinnati College of Medicine
Jeffrey Bednarski, Washington University School of Medicine in St. Louis
Sonali Chaudhury, Northwestern University Feinberg School of Medicine
Theodore B. Moore, UCLA Mattel Children's Hospital
Hilary Haines, The University of Alabama at Birmingham
Blachy J. Dávila Saldaña, The George Washington University School of Medicine and Health Sciences
Deepakbabu Chellapandian, Johns Hopkins All Children's Hospital
Ahmad Rayes, University of Utah School of Medicine
Karin Chen, University of Washington
Emi Caywood, Nemours Children's Health System
Shanmuganathan Chandrakasan, Emory University School of Medicine
Mark Thomas Vander Lugt, University of Michigan, Ann Arbor
Christen Ebens, University of Minnesota Twin Cities
Pierre Teira, CHU Sainte-Justine - Le Centre Hospitalier Universitaire Mère-Enfant
Evan Shereck, Oregon Health & Science University
Holly Miller, Phoenix Children's Hospital
Victor Aquino, UT Southwestern Medical Center
Lolie C. Yu, LSU Health Sciences Center - New OrleansFollow

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Clinical Immunology


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.

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