B 细胞在普通变异性免疫缺陷中的作用。
Role of B cells in common variable immune deficiency.
机构信息
Department of Medicine, Division of Allergy and Immunology, Mount Sinai Medical Center, New York, NY 10029, USA.
出版信息
Expert Rev Clin Immunol. 2009 Sep;5(5):557-64. doi: 10.1586/eci.09.43.
Abstract
Common variable immune deficiency is a heterogeneous immune deficiency characterized by reduced serum immunoglobulins and a lack of antibodies. As the name implies, B-cell defects are variably defective. In particular, peripheral blood isotype-switched CD27(+) memory B cells are reduced in number and have been the basis of several classification schemes. A lack of these B cells has been associated with selected clinical conditions, including immune cytopenias, splenomegaly, granulomatous disease and lymphadenopathy. Genetic defects in ICOS, CD19 and TACI have been described. In addition to defects in the production or survival of memory B cells, in most subjects, B cells have defects in Toll-like receptor signaling.
摘要
普通可变免疫缺陷是一种异质性免疫缺陷，其特征是血清免疫球蛋白减少和缺乏抗体。顾名思义，B 细胞缺陷是可变的。特别是外周血同种型转换的 CD27(+)记忆 B 细胞数量减少，这是几种分类方案的基础。这些 B 细胞的缺乏与某些临床状况有关，包括免疫细胞减少症、脾肿大、肉芽肿病和淋巴结病。已经描述了 ICOS、CD19 和 TACI 中的遗传缺陷。除了记忆 B 细胞的产生或存活缺陷外，在大多数患者中，B 细胞还存在 Toll 样受体信号缺陷。
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