Abstract：
UNASSIGNED: Inborn errors of immunity (IEIs) refer to a heterogeneous category of diseases with defects in the number and/or function of components of the immune system. Immunoglobulin A (IgA) deficiency is the most prevalent IEI characterized by low serum level of IgA and normal serum levels of IgG and/or IgM. Most of the individuals with IgA deficiency are asymptomatic and are only identified through routine laboratory tests. Others may experience a wide range of clinical features including mucosal infections, allergies, and malignancies as the most important features. IgA deficiency is a multi-complex disease, and the exact pathogenesis of it is still unknown.
UNASSIGNED: This review compiles recent research on genetic and epigenetic factors that may contribute to the development of IgA deficiency. These factors include defects in B-cell development, IgA class switch recombination, synthesis, secretion, and the long-term survival of IgA switched memory B cells and plasma cells.
UNASSIGNED: A better and more comprehensive understanding of the cellular pathways involved in IgA deficiency could lead to personalized surveillance and potentially curative strategies for affected patients, especially those with severe symptoms.
UNASSIGNED: This review compiles recent research on genetic and epigenetic factors that may contribute to the development of IgA deficiency. These factors include defects in B-cell development, IgA class switch recombination, synthesis, secretion, and the long-term survival of IgA switched memory B cells and plasma cells.
UNASSIGNED: A better and more comprehensive understanding of the cellular pathways involved in IgA deficiency could lead to personalized surveillance and potentially curative strategies for affected patients, especially those with severe symptoms.
摘要：
■天生的免疫错误（IEI）是指在免疫系统成分的数量和/或功能上存在缺陷的疾病的异质性类别。免疫球蛋白A（IgA）缺乏症是最普遍的IEI，其特征是IgA的血清水平低，IgG和/或IgM的血清水平正常。大多数IgA缺乏症患者都是无症状的，只能通过常规实验室检查进行鉴定。其他人可能会经历广泛的临床特征，包括粘膜感染，过敏,恶性肿瘤是最重要的特征。IgA缺乏是一种复杂的疾病,其确切的发病机制尚不清楚。
■这篇综述汇集了有关可能导致IgA缺乏发展的遗传和表观遗传因素的最新研究。这些因素包括B细胞发育的缺陷，IgA类开关重组，合成，分泌，和长期存活的IgA转换记忆B细胞和浆细胞。
■对与IgA缺乏有关的细胞通路的更好和更全面的了解可能导致对患病患者的个性化监测和潜在的治疗策略。尤其是那些有严重症状的人。
■这篇综述汇集了有关可能导致IgA缺乏发展的遗传和表观遗传因素的最新研究。这些因素包括B细胞发育的缺陷，IgA类开关重组，合成，分泌，和长期存活的IgA转换记忆B细胞和浆细胞。
■对与IgA缺乏有关的细胞通路的更好和更全面的了解可能导致对患病患者的个性化监测和潜在的治疗策略。尤其是那些有严重症状的人。
