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 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 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

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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

All Science Journal Classification (ASJC) codes

Immunology and Allergy
Immunology

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10.1016/j.clim.2024.109942

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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

@article{10763e6931fd46aa97f78f310ba5d01f,

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.",

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

note = "Publisher Copyright: {\textcopyright} 2024 Elsevier Inc.",

year = "2024",

month = apr,

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

language = "English (US)",

volume = "261",

journal = "Clinical Immunology",

issn = "1521-6616",

publisher = "Academic Press Inc.",

}

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, J, 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

TY - JOUR

T1 - Relevance of lymphocyte proliferation to PHA in severe combined immunodeficiency (SCID) and T cell lymphopenia

AU - Abraham, Roshini S.

AU - Basu, Amrita

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

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.

AU - Haddad, Elie

PY - 2024/4

Y1 - 2024/4

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.

UR - http://www.scopus.com/inward/record.url?scp=85185721479&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85185721479&partnerID=8YFLogxK

U2 - 10.1016/j.clim.2024.109942

DO - 10.1016/j.clim.2024.109942

M3 - Article

C2 - 38367737

AN - SCOPUS:85185721479

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

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