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240606s2024 xx |||||o 00| ||eng c |
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|a 10.1016/j.clim.2024.110269
|2 doi
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|a pubmed24n1476.xml
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|a (PII)S1521-6616(24)00378-4
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|a DE-627
|b ger
|c DE-627
|e rakwb
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|a eng
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| 100 |
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|a Lee, Wen-I
|e verfasserin
|4 aut
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|a Clinical features and lymphocyte immunophenotyping analysis in primary immunodeficiency patients with non-transplant lymphoproliferative disorders
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|c 2024
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|a Text
|b txt
|2 rdacontent
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|a ƒaComputermedien
|b c
|2 rdamedia
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|a ƒa Online-Ressource
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|2 rdacarrier
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|a Date Completed 19.07.2024
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|a Date Revised 19.07.2024
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|a published: Print-Electronic
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|a Citation Status MEDLINE
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|a Copyright © 2024 Elsevier Inc. All rights reserved.
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|a 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
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|a Journal Article
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|a CD21-low
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|a Immunophenotyping
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|a Lymphoproliferative disorders (LPD)
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|a Memory cells
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|a Plasamablast B
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|a Primary immunodeficiency diseases (PID)
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|a Senescent T
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|a Transitional B
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|a Huang, Jing-Long
|e verfasserin
|4 aut
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|a Hsieh, Meng-Ying
|e verfasserin
|4 aut
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| 700 |
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|a Chen, Li-Chen
|e verfasserin
|4 aut
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| 700 |
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|a Yeh, Kuo-Wei
|e verfasserin
|4 aut
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| 700 |
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|a Ou, Liang-Shiou
|e verfasserin
|4 aut
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|a Yao, Tsung-Chieh
|e verfasserin
|4 aut
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|a Wu, Chao-Yi
|e verfasserin
|4 aut
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| 700 |
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|a Lin, Syh-Jae
|e verfasserin
|4 aut
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| 700 |
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|a Chen, Shih-Hsiang
|e verfasserin
|4 aut
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|a Jaing, Tang-Her
|e verfasserin
|4 aut
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| 700 |
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|a Liang, Chi-Jou
|e verfasserin
|4 aut
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| 700 |
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|a Kang, Chen-Chen
|e verfasserin
|4 aut
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| 773 |
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|i Enthalten in
|t Clinical immunology (Orlando, Fla.)
|d 1999
|g 265(2024) vom: 31. Juli, Seite 110269
|w (DE-627)NLM098196855
|x 1521-7035
|7 nnns
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| 773 |
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|g volume:265
|g year:2024
|g day:31
|g month:07
|g pages:110269
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|u http://dx.doi.org/10.1016/j.clim.2024.110269
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