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

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

Copyright © 2024 Elsevier Inc. All rights reserved.

Bibliographische Detailangaben
Veröffentlicht in:Clinical immunology (Orlando, Fla.). - 1999. - 265(2024) vom: 31. Juli, Seite 110269
1. Verfasser: Lee, Wen-I (VerfasserIn)
Weitere Verfasser: Huang, Jing-Long, Hsieh, Meng-Ying, Chen, Li-Chen, Yeh, Kuo-Wei, Ou, Liang-Shiou, Yao, Tsung-Chieh, Wu, Chao-Yi, Lin, Syh-Jae, Chen, Shih-Hsiang, Jaing, Tang-Her, Liang, Chi-Jou, Kang, Chen-Chen
Format: Online-Aufsatz
Sprache:English
Veröffentlicht: 2024
Zugriff auf das übergeordnete Werk:Clinical immunology (Orlando, Fla.)
Schlagworte:Journal Article CD21-low Immunophenotyping Lymphoproliferative disorders (LPD) Memory cells Plasamablast B Primary immunodeficiency diseases (PID) Senescent T Transitional B
Beschreibung
Zusammenfassung:Copyright © 2024 Elsevier Inc. All rights reserved.
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
Beschreibung:Date Completed 19.07.2024
Date Revised 19.07.2024
published: Print-Electronic
Citation Status MEDLINE
ISSN:1521-7035
DOI:10.1016/j.clim.2024.110269