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获得性奥门样综合征,一种可被雷帕霉素逆转的新型移植后自身攻击综合征。

Acquired Omenn-like syndrome, a novel posttransplant autoaggression syndrome reversed by rapamycin.

作者信息

Vinh Donald C, Bin Dhuban Khalid, Mason Helen, Lejtenyi Duncan, Jung Sungmi, Sheppard Donald C, Faury Damien, Jabado Nada, Piccirillo Ciriaco A

机构信息

Division of Infectious Diseases, Department of Medicine, McGill University Health Centre, Montreal, QC, Canada.

出版信息

Clin Vaccine Immunol. 2012 Jan;19(1):109-12. doi: 10.1128/CVI.05456-11. Epub 2011 Nov 16.

DOI:10.1128/CVI.05456-11
PMID:22089244
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC3255959/
Abstract

Graft-versus-host disease is uncommon in autologous hematopoietic cell transplantation (HCT) and is typically brief and mild. We report unusual, protracted, and severe Omenn syndrome-like autoaggression following autologous HCT. We identified a profound FOXP3(+) regulatory T cell defect that coincided with hyperinflammatory T cell responses which were reversible with rapamycin in vitro.

摘要

移植物抗宿主病在自体造血细胞移植(HCT)中并不常见,通常病程短暂且症状轻微。我们报告了自体HCT后出现的罕见、迁延且严重的类奥门综合征自身攻击情况。我们发现了一种严重的FOXP3(+)调节性T细胞缺陷,这与高炎症性T细胞反应同时出现,而雷帕霉素在体外可使这些反应逆转。

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Acquired Omenn-like syndrome, a novel posttransplant autoaggression syndrome reversed by rapamycin.获得性奥门样综合征,一种可被雷帕霉素逆转的新型移植后自身攻击综合征。
Clin Vaccine Immunol. 2012 Jan;19(1):109-12. doi: 10.1128/CVI.05456-11. Epub 2011 Nov 16.
2
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引用本文的文献

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Front Immunol. 2022 Apr 5;13:863218. doi: 10.3389/fimmu.2022.863218. eCollection 2022.

本文引用的文献

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FOXP3+ regulatory T cells in the human immune system.FOXP3+ 调节性 T 细胞在人类免疫系统中的作用。
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2
Defect of regulatory T cells in patients with Omenn syndrome.奥姆enn 综合征患者调节性 T 细胞缺陷。
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Reconstitution of self-tolerance after hematopoietic stem cell transplantation.造血干细胞移植后自身耐受的重建。
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5
Rapamycin monotherapy in patients with type 1 diabetes modifies CD4+CD25+FOXP3+ regulatory T-cells.1型糖尿病患者使用雷帕霉素单一疗法可改变CD4+CD25+FOXP3+调节性T细胞。
Diabetes. 2008 Sep;57(9):2341-7. doi: 10.2337/db08-0138. Epub 2008 Jun 16.
6
Lack of iNKT cells in patients with combined immune deficiency due to hypomorphic RAG mutations.由于RAG基因功能减弱突变导致联合免疫缺陷的患者中缺乏不变自然杀伤T细胞。
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7
Induction of autologous graft-versus-host disease: results of a randomized prospective clinical trial in patients with poor risk lymphoma.自体移植物抗宿主病的诱导:对预后不良淋巴瘤患者进行的一项随机前瞻性临床试验结果
Biol Blood Marrow Transplant. 2007 Oct;13(10):1185-91. doi: 10.1016/j.bbmt.2007.06.011. Epub 2007 Aug 3.
8
Rapamycin promotes expansion of functional CD4+CD25+FOXP3+ regulatory T cells of both healthy subjects and type 1 diabetic patients.雷帕霉素可促进健康受试者和1型糖尿病患者功能性CD4+CD25+FOXP3+调节性T细胞的扩增。
J Immunol. 2006 Dec 15;177(12):8338-47. doi: 10.4049/jimmunol.177.12.8338.
9
Omenn syndrome: therapeutic effects of cyclosporin.奥门综合征:环孢素的治疗效果。
J Paediatr Child Health. 2006 May;42(5):319-20. doi: 10.1111/j.1440-1754.2006.00863.x.
10
Immune tolerance to self-major histocompatibility complex class II antigens after bone marrow transplantation: role of regulatory T cells.骨髓移植后对自身主要组织相容性复合体II类抗原的免疫耐受:调节性T细胞的作用
Biol Blood Marrow Transplant. 2006 May;12(5):518-29. doi: 10.1016/j.bbmt.2006.01.007.