Department of Hematology, Tianjin Medical University General Hospital, 154 Anshan St, Heping District, Tianjin, China.
Curr Pharm Des. 2019;25(3):236-241. doi: 10.2174/1381612825666190313113601.
Abnormal activation of the immune system plays an important role in the pathogenesis of aplastic anemia (AA). Various immune cells and cytokines constitute a complex immune network, leading to bone marrow failure. The known pathogenesis is an increase of the myeloid dendritic cell (mDC)/ plasmacytoid dendritic cell (pDC) ratio, which causes the ratio of T helper (Th)1/Th2 to be skewed in favor of Th1 and eventually leads to an abnormal activation of cytotoxic T lymphocyte (CTL). The antigens that stimulate T cells in the context of AA remain unknown. In this process, regulatory T (Treg), Th17, natural killer (NK) cell, memory T cell and negative hematopoietic regulatory factors are also involved. In addition, genetic background (e.g., chromosomal abnormalities, telomere attrition, somatic cell mutations), abnormal bone marrow hematopoietic microenvironment and viral infection may also contribute to the pathogenesis of AA. This review summarizes the recent studies of the pathogenesis of AA and the current status of AA research.
免疫系统的异常激活在再生障碍性贫血（AA）的发病机制中起着重要作用。各种免疫细胞和细胞因子构成了一个复杂的免疫网络，导致骨髓衰竭。已知的发病机制是骨髓源性树突状细胞（mDC）/浆细胞样树突状细胞（pDC）比值增加，导致辅助性 T 细胞（Th）1/Th2 比值向 Th1 倾斜，最终导致细胞毒性 T 淋巴细胞（CTL）异常激活。刺激 AA 中 T 细胞的抗原仍然未知。在这个过程中，调节性 T（Treg）、Th17、自然杀伤（NK）细胞、记忆 T 细胞和负性造血调节因子也参与其中。此外，遗传背景（如染色体异常、端粒损耗、体细胞突变）、异常的骨髓造血微环境和病毒感染也可能导致 AA 的发病机制。本综述总结了 AA 发病机制的最新研究进展和 AA 研究的现状。
