原发性免疫缺陷患者非移植淋巴增生性疾病的临床特征和淋巴细胞免疫分型分析。

IF 4.5 3区 医学 Q2 IMMUNOLOGY Clinical immunology Pub Date : 2024-06-04 DOI:10.1016/j.clim.2024.110269
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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引用次数: 0

摘要

淋巴组织增生性疾病(LPD)是一种异质性疾病,最初被归类为 "免疫调节失调疾病"。2003-2022 年间,96 名台湾患者中有 31 人(中位数为 66 个月,范围为 0.03-675 个月)患上了淋巴增生性疾病,主要包括可触及的淋巴结病(10 人)、与难治性炎症性肠病(IBD)相关的肠道淋巴结病(8 人)以及长期随访期间(中位数为 144 个月,范围为 3-252 个月)出现的肝脾肿大(7 人)。他们分布在抗体缺乏(CVID 2 例、TTC37、PIK3CD、PIK3R1 和 AICDA 各 2 例)、吞噬细胞(CYBB 4 例、STAT1 1 例和 IFNRG1 1 例)、免疫失调(FOXP3 2 例、XIAP 2 例和 HLH 2 例)、联合免疫缺陷(IL2RG.CD40L.ZAP70 和 IL2RG.CD40L.ZAP70 各 2 例)和肝脾肿大等类别;CD40L、ZAP70 和未知)、综合征特征(2 例 STAT3-LOF、1 例 WAS 和 1 例 ATM)以及 3 例抗-IFN-γ 自身抗体。在横断面免疫分型中经常观察到衰老(CD8 + CD57+)和低 CD21、紊乱的过渡 B(CD38 + IgM++)、浆细胞 B(CD38++IgM-)、记忆 B(CD19 + CD27+)和 TEMRA(CD27-IgD-)成分增多,并有发展为 LPD 的趋势。
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Clinical features and lymphocyte immunophenotyping analysis in primary immunodeficiency patients with non-transplant lymphoproliferative disorders

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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来源期刊
Clinical immunology
Clinical immunology 医学-免疫学
CiteScore
12.30
自引率
1.20%
发文量
212
审稿时长
34 days
期刊介绍: Clinical Immunology publishes original research delving into the molecular and cellular foundations of immunological diseases. Additionally, the journal includes reviews covering timely subjects in basic immunology, along with case reports and letters to the editor.
期刊最新文献
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