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 Chen; Emi 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 L 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

doi:10.1016/j.clim.2024.109942

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 L 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

Pediatrics - Blood/Marrow Transplant

Research output: Contribution to journal › Article › peer-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 language	English (US)
Article number	109942
Journal	Clinical Immunology
Volume	261
DOIs	https://doi.org/10.1016/j.clim.2024.109942
State	Published - Apr 2024

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Publisher Copyright:
© 2024 Elsevier Inc.

Keywords

Mitogen
PHA
PIDTC
SCID
Severe combined immunodeficiency
T-cell lymphopenia
T-cell proliferation

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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., ... Haddad, E. (2024). Relevance of lymphocyte proliferation to PHA in severe combined immunodeficiency (SCID) and T cell lymphopenia. Clinical Immunology, 261, Article 109942. https://doi.org/10.1016/j.clim.2024.109942

Abraham, RS, Basu, A, Heimall, JR, Dunn, E, Yip, A, Kapadia, M, Kapoor, N, Satter, LF, Buckley, R, O'Reilly, R, Cuvelier, GDE, Chandra, S, Bednarski, J, Chaudhury, S, Moore, TB, Haines, H, Dávila Saldaña, BJ, Chellapandian, D, Rayes, A, Chen, K, Caywood, E, Chandrakasan, S, Lugt, MTV, Ebens, C, Teira, P, Shereck, E, Miller, H, Aquino, V, Eissa, H, Yu, LC, Gillio, A, Madden, L, Knutsen, A, Shah, AJ, DeSantes, K, Barnum, JL, Broglie, L, Joshi, AY, Kleiner, G, Dara, J, Prockop, S, Martinez, C, Mousallem, T, Oved, J, Burroughs, L, Marsh, R, Torgerson, TR, Leiding, JW, Pai, SY, Kohn, DB, Pulsipher, MA, Griffith, LM, Notarangelo, LD, Cowan, MJ, Puck, J, Dvorak, CC & Haddad, E 2024, 'Relevance of lymphocyte proliferation to PHA in severe combined immunodeficiency (SCID) and T cell lymphopenia', Clinical Immunology, vol. 261, 109942. https://doi.org/10.1016/j.clim.2024.109942

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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.",

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doi = "10.1016/j.clim.2024.109942",

language = "English (US)",

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AU - Heimall, Jennifer R.

AU - Dunn, Elizabeth

AU - Yip, Alison

AU - Kapadia, Malika

AU - Kapoor, Neena

AU - Satter, Lisa Forbes

AU - Buckley, Rebecca

AU - O'Reilly, Richard

AU - Cuvelier, Geoffrey D.E.

AU - Chandra, Sharat

AU - Bednarski, Jeffrey

AU - Chaudhury, Sonali

AU - Moore, Theodore B.

AU - Haines, Hilary

AU - Dávila Saldaña, Blachy J.

AU - Chellapandian, Deepakbabu

AU - Rayes, Ahmad

AU - Chen, Karin

AU - Caywood, Emi

AU - Chandrakasan, Shanmuganathan

AU - Lugt, Mark Thomas Vander

AU - Ebens, Christen

AU - Teira, Pierre

AU - Shereck, Evan

AU - Miller, Holly

AU - Aquino, Victor

AU - Eissa, Hesham

AU - Yu, Lolie C.

AU - Gillio, Alfred

AU - Madden, Lisa

AU - Knutsen, Alan

AU - Shah, Ami J.

AU - DeSantes, Kenneth

AU - Barnum, Jessie L

AU - Broglie, Larisa

AU - Joshi, Avni Y.

AU - Kleiner, Gary

AU - Dara, Jasmeen

AU - Prockop, Susan

AU - Martinez, Caridad

AU - Mousallem, Talal

AU - Oved, Joseph

AU - Burroughs, Lauri

AU - Marsh, Rebecca

AU - Torgerson, Troy R.

AU - Leiding, Jennifer W.

AU - Pai, Sung Yun

AU - Kohn, Donald B.

AU - Pulsipher, Michael A.

AU - Griffith, Linda M.

AU - Notarangelo, Luigi D.

AU - Cowan, Morton J.

AU - Puck, Jennifer

AU - Dvorak, Christopher C.

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N2 - 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.

AB - 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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KW - PHA

KW - PIDTC

KW - SCID

KW - Severe combined immunodeficiency

KW - T-cell lymphopenia

KW - T-cell proliferation

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DO - 10.1016/j.clim.2024.109942

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SN - 1521-6616

VL - 261

JO - Clinical Immunology

JF - Clinical Immunology

M1 - 109942

ER -

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

Abstract

Bibliographical note

Keywords

PubMed: MeSH publication types

Access

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