在免疫/原发性免疫缺陷的先天性错误中异常的免疫球蛋白表达和B细胞毛囊组织
Shachar Naor1, Ginette Schiby1, Etai Adam2
1Department of Pathology, Sheba Medical Center, Ramat Gan, Israel.
Virchows Archiv : an international journal of pathology
|December 29, 2025
概括
具有先天性免疫缺陷 (IEI/PID) 的患者的反应性淋巴腺症可以显示出诊断线索. 在活检中分析B细胞毛囊结构和免疫球蛋白生产可能有助于识别需要免疫评估的患者.
科学领域:
- 免疫学 免疫学 免疫学
- 病理学 病理学 病理学
- 遗传学 是一个遗传学.
背景情况:
- 患有先天性免疫缺陷 (IEI/PID) 的患者经常出现反应性淋巴腺病.
- 淋巴结活检通常用于排除淋巴瘤或感染.
- 在IEI/PID中淋巴组织异常的潜在诊断价值仍未得到充分研究.
研究的目的:
- 为了调查IEI/PID患者的反应性淋巴组织是否含有诊断线索,以了解他们潜在的免疫功能障碍.
- 为了比较IEI/PID患者与对照者的B细胞毛囊结构和免疫球蛋白生产.
- 评估这些发现在识别可能受益于进一步免疫学评估的患者中的有用性.
主要方法:
- 分析B细胞毛囊结构,包括生殖中心 (GC) 存在和T毛囊辅助细胞 (TFH) 透.
- 免疫球蛋白 (Ig) 生产的评估,特别是IgG+类切换 (CS) 与IgM/IgD+非IgG-CS的Ig.
- 对35名IEI/PID患者的国际队列和13名儿科对照组之间的发现进行比较.
主要成果:
- 在45.7%的IEI/PID患者中观察到异常的B细胞毛囊 (缺席/裸体GC,增加的TFH细胞),而对照组则为15.4%.
- 异常的血细胞/血细胞 (PB/PC) 密度 (IgM+,IgD+,IgG+或CD138+) 在79.4%的IEI/PID患者中被观察到,而对照组为7.7%.
- 在42%的IEI/PID患者中存在单独的IgG+ CS PB/PC缺乏,IgG+ PB/PC密度与血清IgG水平之间存在强烈的相关性.
结论:
- B细胞毛囊异常和Ig生产的改变是IEI/PID患者反应性淋巴腺病变的常见发现.
- 这些组织学特征可以作为潜在免疫功能障碍的诊断线索.
- 在淋巴结活检中评估B细胞毛囊结构和Ig生产,可以帮助识别需要免疫评估的患者.
相关概念视频
Humoral Immune Responses
83.1K
Overview
83.1K
Immunodeficiency Diseases
1.9K
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...
1.9K
Development of Immunocompetence
759
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...
759
B Cell Activation and Differentiation
15.7K
The adaptive immune response, a sophisticated defense mechanism, relies on the activation and differentiation of B lymphocytes, or B cells. These processes enable our bodies to mount a tailored response against specific pathogens such as bacteria, free virus particles, toxins, and parasites.
When naive B cells encounter a specific antigen that can bind to the B cell receptor (BCR) on their surface, they undergo sensitization to respond to the antigen's presence. Sensitization begins with...
When naive B cells encounter a specific antigen that can bind to the B cell receptor (BCR) on their surface, they undergo sensitization to respond to the antigen's presence. Sensitization begins with...
15.7K
Cells of the Adaptive Immune Response
8.3K
The T and B lymphocytes of the adaptive immune system develop from common lymphoid progenitor cells in the bone marrow. These progenitors give rise to precursors that eventually develop into both T and B lymphocytes. As these precursors mature, they gain the ability to detect and respond to foreign antigens in the body, a process known as immunocompetence. Additionally, these precursors acquire self-tolerance, a process that ensures they do not react to self-antigens. This intricate system...
8.3K
Primary Lymphoid Organs
14.5K
Primary lymphoid organs are pivotal in the formation, development, and maturation of lymphocytes, the white blood cells that serve as the backbone of our immune system. This crucial function underscores their fundamental role in maintaining our overall health and immunity. The two primary lymphoid organs of prime importance are the red bone marrow and the thymus.
The red bone marrow is a soft, spongy tissue nestled in the interior of long bones such as the humerus and femur. It is the site...
The red bone marrow is a soft, spongy tissue nestled in the interior of long bones such as the humerus and femur. It is the site...
14.5K


