了解不常见的:洞察瘤相关免疫缺陷的洞察力
Xin Rong Lim1, Bernard Pui Lam Leung1,2, Evan Tsien Ming Tan1
1Department of Rheumatology, Allergy and Immunology, Tan Tock Seng Hospital, Singapore.
Asian Pacific journal of allergy and immunology
|February 16, 2025
概括
胸腺瘤相关免疫缺陷 (TAI) 导致严重感染,自身免疫问题和癌症,导致高死亡率. 对于患有这种罕见的成人发病症的患者来说,早期诊断和治疗至关重要.
科学领域:
- 免疫学 免疫学 免疫学
- 在瘤学瘤学.
- 临床医学 临床医学
背景情况:
- 胸腺瘤相关免疫缺陷 (TAI) 是一种罕见的成年期获得性免疫缺陷.
- 它包括经典的古德综合征 (GS) 与胸腺瘤和低血糖球蛋白血症,以及非经典的形式.
- TAI涉及B细胞和T细胞缺乏,导致显著的发病率和死亡率,免疫病理不清楚.
研究的目的:
- 分析21名TAI患者的临床特征,实验室发现,免疫学概况和治疗结果.
- 调查TAI中相关的并发症和并发症.
- 为了探索TAI患者的一个子集中的抗细胞因子抗体.
主要方法:
- 在1999年1月至2023年12月期间诊断的21名TAI患者的回顾性审查.
- 从医疗记录中提取临床,实验室,治疗和结果数据.
- 在七名同意患者中进行抗细胞因子抗体分析.
主要成果:
- TAI主要影响女性 (57.1%),诊断时平均年龄为61.3岁.
- 19名患者患有经典的GS;常见的并发症包括支气管切除 (57.1%),鼻炎 (23.8%) 和其他恶性瘤 (23.8%).
- 经常出现自身免疫性疾病 (47.6%) 和机会性感染;9名患者在平均4.2年内死亡.
结论:
- TAI与严重的发病率和死亡率有关.
- 这种综合症使患者易患机会性感染,自身免疫并发症和恶性瘤.
- 了解TAI的免疫病理学对于改善患者的治疗结果至关重要.
相关概念视频
Immunodeficiency Diseases
895
Immunodeficiency disorders are conditions in which the immune system's ability to fight infectious disease and cancer is compromised or entirely absent. The immune system comprises a complex network of cells, tissues, and organs that work together to protect the body from potentially harmful invaders. When this system is deficient or not functioning properly, it leaves the body susceptible to infections, diseases, or other complications.
There are three main causes of immunodeficiency...
There are three main causes of immunodeficiency...
895
Tumor Immunotherapy
463
Immunotherapy is a treatment that boosts or manipulates the immune system to fight diseases, including cancer. For instance, by stimulating an immune response through vaccinations against viruses that cause cancers, like hepatitis B virus and human papillomavirus, these diseases can be prevented. Nonetheless, some cancer cells can avoid the immune system due to their rapid mutation and division. The immune response to many cancers involves three phases: elimination, equilibrium, and escape.
463
Cell-mediated Immune Responses
66.7K
Overview
66.7K
Development of Immunocompetence
282
The initiation of cell-mediated immunity can be observed as early as the third month of fetal growth, with active antibody-mediated immunity following approximately one month later.
The initial cells that migrate from the fetal thymus settle within the skin and epithelial tissues lining the mouth, digestive tract, and in females, the uterus and vagina. These cells, including skin-based dendritic cells, serve as antigen-presenting cells, playing a key role in T cell activation.
Subsequent T...
The initial cells that migrate from the fetal thymus settle within the skin and epithelial tissues lining the mouth, digestive tract, and in females, the uterus and vagina. These cells, including skin-based dendritic cells, serve as antigen-presenting cells, playing a key role in T cell activation.
Subsequent T...
282
T Cell Types and Functions
897
When T cells with CD4 markers are activated, they give rise to two types of effector cells: helper T cells and regulatory T cells. Meanwhile, T cells with CD8 markers differentiate into effector cytotoxic T cells. The differentiation of CD4 T cells into helper T cell subsets, such as Th1, Th2, and Th17 cells, is dependent on the antigen type, antigen-presenting cell, and regulatory cytokines.
Th1 cells stimulate dendritic cells to express necessary co-stimulatory molecules on their surfaces for...
Th1 cells stimulate dendritic cells to express necessary co-stimulatory molecules on their surfaces for...
897
T Cell Activation and Clonal Selection
629
T cells are integral to our adaptive immune system, recognizing and effectively responding to foreign antigens. T cell activation and clonal selection are pivotal in orchestrating this immune response. This article elucidates these mechanisms, detailing the roles of cluster of differentiation (CD) markers, major histocompatibility complex (MHC) molecules, costimulatory signals, and the process of clonal selection.
Naive T cells that have not yet encountered an antigen express two primary CD...
Naive T cells that have not yet encountered an antigen express two primary CD...
629


