Clinical features and lymphocyte immunophenotyping analysis in primary immunodeficiency patients with non-transplant lymphoproliferative disorders

Wen I. Lee; Jing Long Huang; Meng Ying Hsieh; Li Chen Chen; Kuo Wei Yeh; Liang Shiou Ou; Tsung Chieh Yao; Chao Yi Wu; Syh Jae Lin; Shih Hsiang Chen; Tang Her Jaing; Chi Jou Liang; Chen Chen Kang

doi:10.1016/j.clim.2024.110269

Clinical features and lymphocyte immunophenotyping analysis in primary immunodeficiency patients with non-transplant lymphoproliferative disorders

Wen I. Lee^*, Jing Long Huang, Meng Ying Hsieh, Li Chen Chen, Kuo Wei Yeh, Liang Shiou Ou, Tsung Chieh Yao, Chao Yi Wu, Syh Jae Lin, Shih Hsiang Chen, Tang Her Jaing, Chi Jou Liang, Chen Chen Kang

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1 引文斯高帕斯（Scopus）

摘要

Lymphoproliferative disorders (LPD) comprise a heterogeneous group and are originally classified into the “Disease of immune dysregulation” category. Of 96 Taiwanese patients during 2003–2022, 31 (median 66, range 0.03–675 months) developed LPD, mainly including palpable lymphadenopathy (in 10 patients), intestinal lymphadenopathy associated with refractory inflammatory bowel disease (IBD in 8) and hepatosplenomegaly (in 7) during long-term follow-up (median 144, range 3–252 months). They distributed in the categories of antibody deficiency (2 CVID, 2 TTC37, PIK3CD, PIK3R1 and AICDA each), phagocyte (4 CYBB, 1 STAT1 and 1 IFNRG1), immune dysregulation (2 FOXP3, 2 XIAP and 2 HLH), combined immunodeficiencies (2 IL2RG; CD40L, ZAP70 and unknown each), syndromic features (2 STAT3-LOF, 1 WAS and 1 ATM) and three with anti-IFN-γ autoantibodies. An increased senescent (CD8 + CD57+) and CD21-low, disturbed transitional B (CD38 + IgM++), plasmablast B (CD38++IgM-), memory B (CD19 + CD27+) and T_EMRA (CD27-IgD-) components were often observed in cross-sectional immunophenotyping and trended to develop LPD.

原文	英語
文章編號	110269
頁（從 - 到）	110269
期刊	Clinical Immunology
卷	265
DOIs	https://doi.org/10.1016/j.clim.2024.110269
出版狀態	已出版 - 08 2024
對外發佈	是

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10.1016/j.clim.2024.110269

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Lee, W. I., Huang, J. L., Hsieh, M. Y., Chen, L. C., Yeh, K. W., Ou, L. S., Yao, T. C., Wu, C. Y., Lin, S. J., Chen, S. H., Jaing, T. H., Liang, C. J., & Kang, C. C. (2024). Clinical features and lymphocyte immunophenotyping analysis in primary immunodeficiency patients with non-transplant lymphoproliferative disorders. Clinical Immunology, 265, 110269. 文章 110269. https://doi.org/10.1016/j.clim.2024.110269

@article{67161f93bf914621a5fb9e095f6ab4f8,

title = "Clinical features and lymphocyte immunophenotyping analysis in primary immunodeficiency patients with non-transplant lymphoproliferative disorders",

abstract = "Lymphoproliferative disorders (LPD) comprise a heterogeneous group and are originally classified into the “Disease of immune dysregulation” category. Of 96 Taiwanese patients during 2003–2022, 31 (median 66, range 0.03–675 months) developed LPD, mainly including palpable lymphadenopathy (in 10 patients), intestinal lymphadenopathy associated with refractory inflammatory bowel disease (IBD in 8) and hepatosplenomegaly (in 7) during long-term follow-up (median 144, range 3–252 months). They distributed in the categories of antibody deficiency (2 CVID, 2 TTC37, PIK3CD, PIK3R1 and AICDA each), phagocyte (4 CYBB, 1 STAT1 and 1 IFNRG1), immune dysregulation (2 FOXP3, 2 XIAP and 2 HLH), combined immunodeficiencies (2 IL2RG; CD40L, ZAP70 and unknown each), syndromic features (2 STAT3-LOF, 1 WAS and 1 ATM) and three with anti-IFN-γ autoantibodies. An increased senescent (CD8 + CD57+) and CD21-low, disturbed transitional B (CD38 + IgM++), plasmablast B (CD38++IgM-), memory B (CD19 + CD27+) and TEMRA (CD27-IgD-) components were often observed in cross-sectional immunophenotyping and trended to develop LPD.",

keywords = "CD21-low, Immunophenotyping, Lymphoproliferative disorders (LPD), Memory cells, Plasamablast B, Primary immunodeficiency diseases (PID), Senescent T, Transitional B, Immunologic Deficiency Syndromes/immunology, Humans, Middle Aged, Child, Preschool, Male, Infant, Lymphocytes/immunology, Young Adult, Adolescent, Female, Adult, Lymphoproliferative Disorders/immunology, Child",

author = "Lee, {Wen I.} and Huang, {Jing Long} and Hsieh, {Meng Ying} and Chen, {Li Chen} and Yeh, {Kuo Wei} and Ou, {Liang Shiou} and Yao, {Tsung Chieh} and Wu, {Chao Yi} and Lin, {Syh Jae} and Chen, {Shih Hsiang} and Jaing, {Tang Her} and Liang, {Chi Jou} and Kang, {Chen Chen}",

year = "2024",

month = aug,

doi = "10.1016/j.clim.2024.110269",

language = "英语",

volume = "265",

pages = "110269",

journal = "Clinical Immunology",

issn = "1521-6616",

publisher = "Academic Press Inc.",

}

Lee, WI, Huang, JL, Hsieh, MY, Chen, LC, Yeh, KW, Ou, LS , Yao, TC, Wu, CY, Lin, SJ, Chen, SH, Jaing, TH, Liang, CJ & Kang, CC 2024, 'Clinical features and lymphocyte immunophenotyping analysis in primary immunodeficiency patients with non-transplant lymphoproliferative disorders', Clinical Immunology, 卷 265, 110269, 頁 110269. https://doi.org/10.1016/j.clim.2024.110269

TY - JOUR

T1 - Clinical features and lymphocyte immunophenotyping analysis in primary immunodeficiency patients with non-transplant lymphoproliferative disorders

AU - Lee, Wen I.

AU - Huang, Jing Long

AU - Hsieh, Meng Ying

AU - Chen, Li Chen

AU - Yeh, Kuo Wei

AU - Ou, Liang Shiou

AU - Yao, Tsung Chieh

AU - Wu, Chao Yi

AU - Lin, Syh Jae

AU - Chen, Shih Hsiang

AU - Jaing, Tang Her

AU - Liang, Chi Jou

AU - Kang, Chen Chen

PY - 2024/8

Y1 - 2024/8

N2 - Lymphoproliferative disorders (LPD) comprise a heterogeneous group and are originally classified into the “Disease of immune dysregulation” category. Of 96 Taiwanese patients during 2003–2022, 31 (median 66, range 0.03–675 months) developed LPD, mainly including palpable lymphadenopathy (in 10 patients), intestinal lymphadenopathy associated with refractory inflammatory bowel disease (IBD in 8) and hepatosplenomegaly (in 7) during long-term follow-up (median 144, range 3–252 months). They distributed in the categories of antibody deficiency (2 CVID, 2 TTC37, PIK3CD, PIK3R1 and AICDA each), phagocyte (4 CYBB, 1 STAT1 and 1 IFNRG1), immune dysregulation (2 FOXP3, 2 XIAP and 2 HLH), combined immunodeficiencies (2 IL2RG; CD40L, ZAP70 and unknown each), syndromic features (2 STAT3-LOF, 1 WAS and 1 ATM) and three with anti-IFN-γ autoantibodies. An increased senescent (CD8 + CD57+) and CD21-low, disturbed transitional B (CD38 + IgM++), plasmablast B (CD38++IgM-), memory B (CD19 + CD27+) and TEMRA (CD27-IgD-) components were often observed in cross-sectional immunophenotyping and trended to develop LPD.

AB - Lymphoproliferative disorders (LPD) comprise a heterogeneous group and are originally classified into the “Disease of immune dysregulation” category. Of 96 Taiwanese patients during 2003–2022, 31 (median 66, range 0.03–675 months) developed LPD, mainly including palpable lymphadenopathy (in 10 patients), intestinal lymphadenopathy associated with refractory inflammatory bowel disease (IBD in 8) and hepatosplenomegaly (in 7) during long-term follow-up (median 144, range 3–252 months). They distributed in the categories of antibody deficiency (2 CVID, 2 TTC37, PIK3CD, PIK3R1 and AICDA each), phagocyte (4 CYBB, 1 STAT1 and 1 IFNRG1), immune dysregulation (2 FOXP3, 2 XIAP and 2 HLH), combined immunodeficiencies (2 IL2RG; CD40L, ZAP70 and unknown each), syndromic features (2 STAT3-LOF, 1 WAS and 1 ATM) and three with anti-IFN-γ autoantibodies. An increased senescent (CD8 + CD57+) and CD21-low, disturbed transitional B (CD38 + IgM++), plasmablast B (CD38++IgM-), memory B (CD19 + CD27+) and TEMRA (CD27-IgD-) components were often observed in cross-sectional immunophenotyping and trended to develop LPD.

KW - CD21-low

KW - Immunophenotyping

KW - Lymphoproliferative disorders (LPD)

KW - Memory cells

KW - Plasamablast B

KW - Primary immunodeficiency diseases (PID)

KW - Senescent T

KW - Transitional B

KW - Immunologic Deficiency Syndromes/immunology

KW - Humans

KW - Middle Aged

KW - Child, Preschool

KW - Male

KW - Infant

KW - Lymphocytes/immunology

KW - Young Adult

KW - Adolescent

KW - Female

KW - Adult

KW - Lymphoproliferative Disorders/immunology

KW - Child

UR - http://www.scopus.com/inward/record.url?scp=85195366858&partnerID=8YFLogxK

U2 - 10.1016/j.clim.2024.110269

DO - 10.1016/j.clim.2024.110269

M3 - 文章

C2 - 38838929

AN - SCOPUS:85195366858

SN - 1521-6616

VL - 265

SP - 110269

JO - Clinical Immunology

JF - Clinical Immunology

M1 - 110269

ER -

Clinical features and lymphocyte immunophenotyping analysis in primary immunodeficiency patients with non-transplant lymphoproliferative disorders

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