Division of Allergy and Immunology, Ann and Robert H. Lurie Children's Hospital of Chicago, Chicago, IL 60611, USA.
Department of Pediatrics, Northwestern University, Chicago, IL 60611, USA.
Cells. 2022 Oct 24;11(21):3353. doi: 10.3390/cells11213353.
B cells and antibodies are indispensable for host immunity. Our understanding of the mechanistic processes that underpin how B cells operate has left an indelible mark on the field of clinical pathology, and recently has also dramatically reshaped the therapeutic landscape of diseases that were once considered incurable. Evaluating patients with primary immunodeficiency diseases (PID)/inborn errors of immunity (IEI) that primarily affect B cells, offers us an opportunity to further our understanding of how B cells develop, mature, function and, in certain instances, cause further disease. In this review we provide a brief compendium of IEI that principally affect B cells at defined stages of their developmental pathway, and also attempt to offer some educated viewpoints on how the management of these disorders could evolve over the years.
B 细胞和抗体对于宿主免疫至关重要。我们对支撑 B 细胞运作的机制过程的理解，给临床病理学领域留下了不可磨灭的印记，最近也极大地改变了曾经被认为无法治愈的疾病的治疗格局。评估主要影响 B 细胞的原发性免疫缺陷病 (PID)/先天性免疫缺陷 (IEI) 患者，使我们有机会进一步了解 B 细胞如何发育、成熟、发挥功能，以及在某些情况下导致进一步的疾病。在这篇综述中，我们简要概述了主要影响 B 细胞在其发育途径特定阶段的 IEI，并试图就这些疾病的多年来的管理方法提供一些有根据的观点。
