Clinical immunology最新文献

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NLRP12-associated autoinflammatory disease: A novel causal mutation and bioinformatics analyses NLRP12相关自身炎症性疾病:一种新的致病突变和生物信息学分析
IF 8.6 3区 医学
Clinical immunology Pub Date : 2024-06-13 DOI: 10.1016/j.clim.2024.110278
Zhonghua Li , Qi Zhi , Jiahuang Li , Bo Zhu
{"title":"NLRP12-associated autoinflammatory disease: A novel causal mutation and bioinformatics analyses","authors":"Zhonghua Li ,&nbsp;Qi Zhi ,&nbsp;Jiahuang Li ,&nbsp;Bo Zhu","doi":"10.1016/j.clim.2024.110278","DOIUrl":"https://doi.org/10.1016/j.clim.2024.110278","url":null,"abstract":"<div><p>Nucleotide-binding leucine-rich repeat-containing receptor 12-associated autoinflammatory disease (<em>NLRP12</em>-AID) is a rare autosomal dominant disorder. In this study, we reported a case of this rare disease with a novel <em>NLRP12</em> mutation (A218V, rs749659859). The patient displayed typical symptoms, including recurrent fever, arthralgia, and skin allergies. Elevated serum IgE, decreased apolipoprotein A1, high-density lipoprotein cholesterol, and fluctuating levels of various leukocyte subtypes, procalcitonin, IL6, creatine kinase, and 25-hydroxyvitamin D were also detected. Inflammatory lesions were observed in multiple organs using <sup>18</sup>F-FDG PET/CT. By mining single-cell transcriptome data, we identified relatively high expression of <em><em>NLRP12</em></em> in monocytes compared to other human peripheral blood mononuclear cells. <em>NLRP12</em>-positive monocytes exhibited reduced expression of <em>IL18</em>, <em>CCL3</em>, and <em>TNFA</em> compared to <em>NLRP12</em>-negative monocytes. Structural analyses suggested that the A218V mutation, along with A218T and F402L, may reduce the ATP-binding affinity of the NLRP12 protein. These findings may provide new insights into the mechanisms of <em>NLRP12</em>-AID, and suggest the potential ATP-based therapy for further investigation.</p></div>","PeriodicalId":10392,"journal":{"name":"Clinical immunology","volume":"265 ","pages":"Article 110278"},"PeriodicalIF":8.6,"publicationDate":"2024-06-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141325179","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
T cell subset composition differs between blood and cerebrospinal fluid in amyotrophic lateral sclerosis 肌萎缩性脊髓侧索硬化症患者血液和脑脊液中的 T 细胞亚群组成不同。
IF 8.6 3区 医学
Clinical immunology Pub Date : 2024-06-07 DOI: 10.1016/j.clim.2024.110270
Solmaz Yazdani , Anikó Lovik , Christina Seitz , Caroline Ingre , Fang Fang , John Andersson
{"title":"T cell subset composition differs between blood and cerebrospinal fluid in amyotrophic lateral sclerosis","authors":"Solmaz Yazdani ,&nbsp;Anikó Lovik ,&nbsp;Christina Seitz ,&nbsp;Caroline Ingre ,&nbsp;Fang Fang ,&nbsp;John Andersson","doi":"10.1016/j.clim.2024.110270","DOIUrl":"10.1016/j.clim.2024.110270","url":null,"abstract":"<div><p>Inflammation is a hallmark of amyotrophic lateral sclerosis (ALS) and is often assessed through biological samples. Due to the easier access, peripheral blood is more commonly phenotyped instead of cerebrospinal fluid (CSF) or affected tissues in ALS. Here, using flow cytometry, we compared the composition of T cell subsets in blood and CSF in ALS patients. We found consistent but weak correlations between blood and CSF for all T cell subsets examined. This finding implies that blood and CSF offer complementary information when characterizing T cell immunity in ALS and blood may not be used as a surrogate for CSF.</p></div>","PeriodicalId":10392,"journal":{"name":"Clinical immunology","volume":"265 ","pages":"Article 110270"},"PeriodicalIF":8.6,"publicationDate":"2024-06-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141295700","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Exploring the HLA complex in autoimmunity: From the risk haplotypes to the modulation of expression 探索自身免疫中的 HLA 复合物:从风险单倍型到表达调节。
IF 8.6 3区 医学
Clinical immunology Pub Date : 2024-06-06 DOI: 10.1016/j.clim.2024.110266
Silvia Sartoris , Giovanna Del Pozzo
{"title":"Exploring the HLA complex in autoimmunity: From the risk haplotypes to the modulation of expression","authors":"Silvia Sartoris ,&nbsp;Giovanna Del Pozzo","doi":"10.1016/j.clim.2024.110266","DOIUrl":"10.1016/j.clim.2024.110266","url":null,"abstract":"<div><p>The genes mapping at the HLA region show high density, strong linkage disequilibrium and high polymorphism, which affect the association of HLA class I and class II genes with autoimmunity. We focused on the HLA haplotypes, genomic structures consisting of an array of specific alleles showing some degrees of genetic association with different autoimmune disorders. GWASs in many pathologies have identified variants in either the coding loci or the flanking regulatory regions, both in linkage disequilibrium in haplotypes, that are frequently associated with increased risk and may influence gene expression. We discuss the relevance of the HLA gene expression because the level of surface heterodimers determines the number of complexes presenting self-antigen and, thus, the strength of pathogenic autoreactive T cells immune response.</p></div>","PeriodicalId":10392,"journal":{"name":"Clinical immunology","volume":"265 ","pages":"Article 110266"},"PeriodicalIF":8.6,"publicationDate":"2024-06-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S1521661624003759/pdfft?md5=7cd06c6f943cee63d821e9d1f86f0600&pid=1-s2.0-S1521661624003759-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141293224","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Clinical features and lymphocyte immunophenotyping analysis in primary immunodeficiency patients with non-transplant lymphoproliferative disorders 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 8.6 3区 医学
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
{"title":"Clinical features and lymphocyte immunophenotyping analysis in primary immunodeficiency patients with non-transplant lymphoproliferative disorders","authors":"Wen-I Lee ,&nbsp;Jing-Long Huang ,&nbsp;Meng-Ying Hsieh ,&nbsp;Li-Chen Chen ,&nbsp;Kuo-Wei Yeh ,&nbsp;Liang-Shiou Ou ,&nbsp;Tsung-Chieh Yao ,&nbsp;Chao-Yi Wu ,&nbsp;Syh-Jae Lin ,&nbsp;Shih-Hsiang Chen ,&nbsp;Tang-Her Jaing ,&nbsp;Chi-Jou Liang ,&nbsp;Chen-Chen Kang","doi":"10.1016/j.clim.2024.110269","DOIUrl":"10.1016/j.clim.2024.110269","url":null,"abstract":"<div><p>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 <em>TTC37</em>, <em>PIK3CD</em>, <em>PIK3R1</em> and <em>AICDA</em> each), phagocyte (4 <em>CYBB</em>, 1 <em>STAT1</em> and 1 <em>IFNRG1</em>), immune dysregulation (2 <em>FOXP3</em>, 2 <em>XIAP</em> and 2 HLH), combined immunodeficiencies (2 <em>IL2RG</em>; <em>CD40L</em>, <em>ZAP70</em> and unknown each), syndromic features (2 <em>STAT3</em>-LOF, 1 <em>WAS</em> and 1 <em>ATM</em>) 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<sub>EMRA</sub> (CD27-IgD-) components were often observed in cross-sectional immunophenotyping and trended to develop LPD.</p></div>","PeriodicalId":10392,"journal":{"name":"Clinical immunology","volume":"265 ","pages":"Article 110269"},"PeriodicalIF":8.6,"publicationDate":"2024-06-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141261210","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Two novel compound heterozygous loss-of-function mutations cause fetal IRAK-4 deficiency presenting with Pseudomonas Aeruginosa sepsis 两种新型复合杂合功能缺失突变导致胎儿IRAK-4缺乏症,并伴有绿脓杆菌败血症。
IF 8.6 3区 医学
Clinical immunology Pub Date : 2024-06-03 DOI: 10.1016/j.clim.2024.110268
Fang Zhang , Zhiwei Wang , Shuai Men, Jinglu Zhang, Leilei Wang
{"title":"Two novel compound heterozygous loss-of-function mutations cause fetal IRAK-4 deficiency presenting with Pseudomonas Aeruginosa sepsis","authors":"Fang Zhang ,&nbsp;Zhiwei Wang ,&nbsp;Shuai Men,&nbsp;Jinglu Zhang,&nbsp;Leilei Wang","doi":"10.1016/j.clim.2024.110268","DOIUrl":"10.1016/j.clim.2024.110268","url":null,"abstract":"<div><h3>Purpose</h3><p>To report a case of a five-month-old Chinese infant who died of interleukin-1 receptor-associated kinase-4 (IRAK-4) deficiency presenting with rapid and progressive <em>Pseudomonas aeruginosa</em> sepsis.</p></div><div><h3>Methods</h3><p>The genetic etiology of IRAK-4 deficiency was confirmed through trio-whole exome sequencing and Sanger sequencing. Functional consequences were invested using an in vitro minigene splicing assay.</p></div><div><h3>Results</h3><p>Trio-whole exome sequencing of genomic DNA identified two novel compound heterozygous mutations, <em>IRAK-4</em> (NM_016123.3): c.942–1G &gt; A and c.644_651+ 6delTTGCAGCAGTAAGT in the proband, which originated from his symptom-free parents. These mutations were predicted to cause frameshifts and generate three truncated proteins without enzyme activity.</p></div><div><h3>Conclusions</h3><p>Our findings expand the range of IRAK-4 mutations and provide functional support for the pathogenic effects of splice-site mutations. Additionally, this case highlights the importance of considering the underlying genetic defects of immunity when dealing with unusually overwhelming infections in previously healthy children and emphasizes the necessity for timely treatment with wide-spectrum antimicrobials.</p></div>","PeriodicalId":10392,"journal":{"name":"Clinical immunology","volume":"265 ","pages":"Article 110268"},"PeriodicalIF":8.6,"publicationDate":"2024-06-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141261220","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Imbalance of SARS-CoV-2-specific CCR6+ and CXCR3+ CD4+ T cells and IFN-γ + CD8+ T cells in patients with Long-COVID 长COVID患者中SARS-CoV-2特异性CCR6+和CXCR3+ CD4+T细胞与IFN-γ + CD8+T细胞的失衡。
IF 8.6 3区 医学
Clinical immunology Pub Date : 2024-05-31 DOI: 10.1016/j.clim.2024.110267
Pedro Martínez-Fleta , María Celeste Marcos , Daniel Jimenez-Carretero , José María Galván-Román , Rosa María Girón-Moreno , Ana Adela Calero-García , Ana Arcos-García , Enrique Martín-Gayo , Hortensia de la Fuente , Laura Esparcia-Pinedo , Javier Aspa , Julio Ancochea , Arantzazu Alfranca , Francisco Sánchez-Madrid
{"title":"Imbalance of SARS-CoV-2-specific CCR6+ and CXCR3+ CD4+ T cells and IFN-γ + CD8+ T cells in patients with Long-COVID","authors":"Pedro Martínez-Fleta ,&nbsp;María Celeste Marcos ,&nbsp;Daniel Jimenez-Carretero ,&nbsp;José María Galván-Román ,&nbsp;Rosa María Girón-Moreno ,&nbsp;Ana Adela Calero-García ,&nbsp;Ana Arcos-García ,&nbsp;Enrique Martín-Gayo ,&nbsp;Hortensia de la Fuente ,&nbsp;Laura Esparcia-Pinedo ,&nbsp;Javier Aspa ,&nbsp;Julio Ancochea ,&nbsp;Arantzazu Alfranca ,&nbsp;Francisco Sánchez-Madrid","doi":"10.1016/j.clim.2024.110267","DOIUrl":"10.1016/j.clim.2024.110267","url":null,"abstract":"<div><p>Long-COVID (LC) is characterised by persistent symptoms for at least 3 months after acute infection. A dysregulation of the immune system and a persistent hyperinflammatory state may cause LC. LC patients present differences in activation and exhaustion states of innate and adaptive compartments. Different T CD4<sup>+</sup> cell subsets can be identified by differential expression of chemokine receptors (CCR). However, changes in T cells with expression of CCRs such as CCR6 and CXCR3 and their relationship with CD8<sup>+</sup> T cells remains unexplored in LC. Here, we performed unsupervised analysis and found CCR6<sup>+</sup> CD4<sup>+</sup> subpopulations enriched in COVID-19 convalescent individuals upon activation with SARS-CoV-2 peptides. SARS-CoV-2 specific CCR6<sup>+</sup> CD4<sup>+</sup> are decreased in LC patients, whereas CXCR3<sup>+</sup> CCR6<sup>-</sup> and CCR4<sup>+</sup> CCR6<sup>-</sup> CD4<sup>+</sup> T cells are increased. LC patients showed lower IFN-γ-secreting CD8<sup>+</sup> T cells after stimulation with SARS-CoV-2 Spike protein. This work underscores the role of CCR6 in the pathophysiology of LC.</p></div>","PeriodicalId":10392,"journal":{"name":"Clinical immunology","volume":"264 ","pages":"Article 110267"},"PeriodicalIF":8.6,"publicationDate":"2024-05-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141199517","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
The immunomodulatory of interleukin-33 in rheumatoid arthritis: A systematic review 白细胞介素-33 对类风湿性关节炎的免疫调节作用:系统综述。
IF 8.6 3区 医学
Clinical immunology Pub Date : 2024-05-31 DOI: 10.1016/j.clim.2024.110264
Renli Liu, Fangfang Wang, Xing Luo, Fengfan Yang, Jie Gao, Haomiao Shen, Zhaohui Zheng
{"title":"The immunomodulatory of interleukin-33 in rheumatoid arthritis: A systematic review","authors":"Renli Liu,&nbsp;Fangfang Wang,&nbsp;Xing Luo,&nbsp;Fengfan Yang,&nbsp;Jie Gao,&nbsp;Haomiao Shen,&nbsp;Zhaohui Zheng","doi":"10.1016/j.clim.2024.110264","DOIUrl":"10.1016/j.clim.2024.110264","url":null,"abstract":"<div><p>Rheumatoid arthritis (RA) is a systemic chronic autoimmune disease that primarily affects the joints and surrounding soft tissues, characterized by chronic inflammation and proliferation of the synovium. Various immune cells are involved in the pathophysiology of RA. The complex interplay of factors such as chronic inflammation, genetic susceptibility, dysregulation of serum antibody levels, among others, contribute to the complexity of the disease mechanism, disease activity, and treatment of RA. Recently, the cytokine storm leading to increased disease activity in RA has gained significant attention. Interleukin-33 (IL-33), a member of the IL-1 family, plays a crucial role in inflammation and immune regulation. ST2 (suppression of tumorigenicity 2 receptor), the receptor for IL-33, is widely expressed on the surface of various immune cells. When IL-33 binds to its receptor ST2, it activates downstream signaling pathways to exert immunoregulatory effects. In RA, IL-33 regulates the progression of the disease by modulating immune cells such as circulating monocytes, tissue-resident macrophages, synovial fibroblasts, mast cells, dendritic cells, neutrophils, T cells, B cells, endothelial cells, and others. We have summarized and analyzed these findings to elucidate the pathways through which IL-33 regulates RA. Furthermore, IL-33 has been detected in the synovium, serum, and synovial fluid of RA patients. Due to inconsistent research results, we conducted a meta-analysis on the association between serum IL-33, synovial fluid IL-33, and the risk of developing RA in patients. The pooled SMD was 1.29 (95% CI: 1.15–1.44), indicating that IL-33 promotes the onset and pathophysiological progression of RA. Therefore, IL-33 may serve as a biomarker for predicting the risk of developing RA and treatment outcomes. As existing drugs for RA still cannot address drug resistance in some patients, new therapeutic approaches are needed to alleviate the significant burden on RA patients and healthcare systems. In light of this, we analyzed the potential of targeting the IL-33/ST2-related signaling pathway to modulate immune cells associated with RA and alleviate inflammation. We also reviewed IL-33 and RA susceptibility-related single nucleotide polymorphisms, suggesting potential involvement of IL-33 and macrophage-related drug-resistant genes in RA resistance therapy. Our review elucidates the role of IL-33 in the pathophysiology of RA, offering new insights for the treatment of RA.</p></div>","PeriodicalId":10392,"journal":{"name":"Clinical immunology","volume":"265 ","pages":"Article 110264"},"PeriodicalIF":8.6,"publicationDate":"2024-05-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141199519","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Allergic bronchopulmonary aspergillosis in a lung transplant recipient treated with mepolizumab 一名肺移植受者在接受甲泼尼珠单抗治疗后出现过敏性支气管肺曲霉病。
IF 8.6 3区 医学
Clinical immunology Pub Date : 2024-05-25 DOI: 10.1016/j.clim.2024.110265
Foteini Ioakeim , Christophe Abellan , Alessio Casutt , Benoit Lechartier , Leslie Noirez , Catherine Beigelman-Aubry , John-David Aubert , Zisis Balmpouzis , Angela Koutsokera
{"title":"Allergic bronchopulmonary aspergillosis in a lung transplant recipient treated with mepolizumab","authors":"Foteini Ioakeim ,&nbsp;Christophe Abellan ,&nbsp;Alessio Casutt ,&nbsp;Benoit Lechartier ,&nbsp;Leslie Noirez ,&nbsp;Catherine Beigelman-Aubry ,&nbsp;John-David Aubert ,&nbsp;Zisis Balmpouzis ,&nbsp;Angela Koutsokera","doi":"10.1016/j.clim.2024.110265","DOIUrl":"10.1016/j.clim.2024.110265","url":null,"abstract":"<div><p>Allergic bronchopulmonary aspergillosis (ABPA) is a complex hypersensitivity reaction to <em>Aspergillus</em> spp. ABPA diagnosis may be challenging due to its non-specific presentation. Standard ABPA treatment consists of systemic corticosteroids and antifungal agents. Mepolizumab, a monoclonal antibody against interleukin-5 seems to be a promising treatment for ABPA. Data about ABPA following lung transplantation (LuTx) are scarce. LuTx recipients are at higher risk for adverse effects of ABPA treatment compared to the general population.</p><p>Here we present a case of a LuTx recipient who was successfully treated with mepolizumab for ABPA following LuTx. Prolonged administration of high dose prednisone was thus avoided. To our knowledge, this is the first case describing mepolizumab administration following LuTx. Mepolizumab seems particularly attractive as a corticosteroid-sparing agent or as an alternative option to antifungal treatments, because of its excellent safety profile and low risk of drug interactions.</p></div>","PeriodicalId":10392,"journal":{"name":"Clinical immunology","volume":"264 ","pages":"Article 110265"},"PeriodicalIF":8.6,"publicationDate":"2024-05-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141157536","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Immune reconstitution and survival, following hematopoietic stem cell transplantation in Omani patients with inborn errors of immunity 阿曼先天性免疫错误患者造血干细胞移植后的免疫重建和存活率
IF 8.6 3区 医学
Clinical immunology Pub Date : 2024-05-23 DOI: 10.1016/j.clim.2024.110263
Salem Al-Tamemi , Abdulhakim Al-Rawas , Murtadha Al-Khabori , Khalil Al-Farsi , Mohammed Al-Huneini , Amr Abdalla , Salam Al-Kindi , David Dennison
{"title":"Immune reconstitution and survival, following hematopoietic stem cell transplantation in Omani patients with inborn errors of immunity","authors":"Salem Al-Tamemi ,&nbsp;Abdulhakim Al-Rawas ,&nbsp;Murtadha Al-Khabori ,&nbsp;Khalil Al-Farsi ,&nbsp;Mohammed Al-Huneini ,&nbsp;Amr Abdalla ,&nbsp;Salam Al-Kindi ,&nbsp;David Dennison","doi":"10.1016/j.clim.2024.110263","DOIUrl":"10.1016/j.clim.2024.110263","url":null,"abstract":"<div><h3>Background</h3><p>Hematopoietic stem cell transplantation (HSCT) is a curative treatment for certain inborn errors of immunity.</p></div><div><h3>Methods</h3><p>A 17-year retrospective cohort study was conducted on 40 immunodeficient patients who underwent HSCT.</p></div><div><h3>Results</h3><p>The median age at transplant was 11.0 months (4.6–61.0). Donors were primarily matched sibling donors (60%). 90% and 85% of patients received conditioning and graft-versus-host disease (GVHD) prophylaxis, respectively. The mean donor chimerism at the last follow-up was 88.6% ± 17.9% (40–100). Median serum immunoglobulin (Ig) G level, CD4+ T-cell count, and CD19+ B-cell count were 11.7 g/L (9.2–13.6), 0.9 × 10<sup>9</sup>/L 0.6–1.2), and 0.5 × 10<sup>9</sup>/L (0.2–0.7), respectively. 29 patients (72.5%) received intravenous immunoglobulins (IVIG) therapy, with a median duration of 10.0 months (4.0–14.0). The median post-transplant follow-up was 6.5 years (IQR:1.4–11.5). The 10-year overall probability of survival is 84.3%.</p></div><div><h3>Conclusion</h3><p>Monitoring IRC is important in ensuring adequate disease-free survival.</p></div>","PeriodicalId":10392,"journal":{"name":"Clinical immunology","volume":"264 ","pages":"Article 110263"},"PeriodicalIF":8.6,"publicationDate":"2024-05-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141141525","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Effects of anti-CD20 therapy on circulating and intrathecal follicular helper T cell subsets in multiple sclerosis 抗CD20疗法对多发性硬化症患者循环和鞘内滤泡辅助T细胞亚群的影响。
IF 8.6 3区 医学
Clinical immunology Pub Date : 2024-05-23 DOI: 10.1016/j.clim.2024.110262
Sahla El Mahdaoui , Marie Mathilde Hansen , Malene Bredahl Hansen , Victoria Hyslop Hvalkof , Helle Bach Søndergaard , Mie Reith Mahler , Jeppe Romme Christensen , Finn Sellebjerg , Marina Rode von Essen
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