Relevance of lymphocyte proliferation to PHA in severe combined immunodeficiency (SCID) and T cell lymphopenia Journal Article
| Authors: | Abraham, R. S.; Basu, A.; Heimall, J. R.; Dunn, E.; Yip, A.; Kapadia, M.; Kapoor, N.; Satter, L. F.; Buckley, R.; O'Reilly, R.; Cuvelier, G. D. E.; Chandra, S.; Bednarski, J.; Chaudhury, S.; Moore, T. B.; Haines, H.; Dávila Saldaña, B. J.; Chellapandian, D.; Rayes, A.; Chen, K.; Caywood, E.; Chandrakasan, S.; Lugt, M. T. V.; Ebens, C.; Teira, P.; Shereck, E.; Miller, H.; Aquino, V.; Eissa, H.; Yu, L. C.; Gillio, A.; Madden, L.; Knutsen, A.; Shah, A. J.; DeSantes, K.; Barnum, J.; Broglie, L.; Joshi, A. Y.; Kleiner, G.; Dara, J.; Prockop, S.; Martinez, C.; Mousallem, T.; Oved, J.; Burroughs, L.; Marsh, R.; Torgerson, T. R.; Leiding, J. W.; Pai, S. Y.; Kohn, D. B.; Pulsipher, M. A.; Griffith, L. M.; Notarangelo, L. D.; Cowan, M. J.; Puck, J.; Dvorak, C. C.; Haddad, E. |
| Article Title: | Relevance of lymphocyte proliferation to PHA in severe combined immunodeficiency (SCID) and T cell lymphopenia |
| 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. © 2024 Elsevier Inc. |
| Keywords: | controlled study; treatment outcome; unclassified drug; human cell; major clinical study; comparative study; flow cytometry; cd3 antigen; cell proliferation; lymphocyte proliferation; t lymphocyte; clinical assessment; analytic method; genotype; lymphocytopenia; dna; radioactivity; data analysis; cd4 lymphocyte count; rag1 protein; rag2 protein; cd3+ t lymphocyte; severe combined immunodeficiency; protein kinase zap 70; interleukin 2 receptor gamma; interleukin 7 receptor alpha; mitogen; janus kinase 3; phytohemagglutinin; human; article; omenn syndrome; t-cell proliferation; scid; hemifacial atrophy; receptor type tyrosine protein phosphatase c; dclre1c protein; pha; pidtc; t-cell lymphopenia; doxribtimine; t cell lymphopenia |
| Journal Title: | Clinical Immunology |
| Volume: | 261 |
| ISSN: | 1521-6616 |
| Publisher: | Elsevier Inc. |
| Date Published: | 2024-04-01 |
| Start Page: | 109942 |
| Language: | English |
| DOI: | 10.1016/j.clim.2024.109942 |
| PUBMED: | 38367737 |
| PROVIDER: | scopus |
| PMCID: | PMC11018339 |
| DOI/URL: | |
| Notes: | Article -- Source: Scopus |
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