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抗体缺陷障碍(存档)

Antibody Deficiency Disorder (Archived)

作者信息

Justiz Vaillant Angel A., Ramphul Kamleshun

机构信息

University of the West Indies

Shanghai Jiao Tong University

Abstract

Antibodies or immunoglobulins (Ig) play an important role in the immune system's mechanisms of defense. They fight off extracellular pathogens, for instance, bacteria, and can neutralize viruses when they are in the bloodstream and other body fluids. Normal individuals have 5 classes of immunoglobulins, which are IgM, IgG, IgA, IgD, and IgE, and immunoglobulin subclasses including IgG1, IgG2, IgG3, IgG4, IgA1, and IgA2. Sometimes an antibody deficiency disorder takes place. It can happen due to several causes, including a genetic absence of an important enzyme in B-cell development that renders immature B cells that are unable to proliferate into mature Ig-producing B cells or may be caused when T lymphocytes do not signal B lymphocytes or are idiopathic.  The most common antibody deficiency disorders include: X-linked agammaglobulinemia (Bruton disease). Transient hypogammaglobulinemia of newborn. Selective Ig immunodeficiencies, for example, IgA selective deficiency. Super IgM syndrome. Common variable immunodeficiency disorder.

摘要

抗体或免疫球蛋白(Ig)在免疫系统的防御机制中发挥着重要作用。它们能抵御细胞外病原体,例如细菌,并且当病毒存在于血液和其他体液中时,能将其中和。正常个体有5类免疫球蛋白,即IgM、IgG、IgA、IgD和IgE,以及免疫球蛋白亚类,包括IgG1、IgG2、IgG3、IgG4、IgA1和IgA2。有时会发生抗体缺乏症。其发生可能有多种原因,包括B细胞发育过程中一种重要酶的基因缺失,这使得未成熟的B细胞无法增殖为产生Ig的成熟B细胞,或者可能是由于T淋巴细胞未向B淋巴细胞发出信号,或是病因不明所致。最常见的抗体缺乏症包括:X连锁无丙种球蛋白血症(布鲁顿病)、新生儿暂时性低丙种球蛋白血症、选择性Ig免疫缺陷,例如IgA选择性缺乏、高IgM综合征、常见变异型免疫缺陷病。

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