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