Chronic lymphocytic leukaemia.
Chronic lymphocytic leukaemia.
复制标题
DOI：
10.1038/nrdp.2016.96
复制
发表时间：
2017-01-19
期刊：
影响因子：
--
通讯作者：
Rai K
中科院分区：
文献类型：
--
作者：
Kipps TJ;Stevenson FK;Wu CJ;Croce CM;Packham G;Wierda WG;O'Brien S;Gribben J;Rai K
慢性淋巴细胞白血病（CLL）是一种CD5+ B细胞的恶性肿瘤，其特征是在血液、骨髓和淋巴组织中积累小的、成熟的淋巴细胞。除了CLL细胞与其他细胞类型（如基质细胞、T细胞和淋巴结中的护士样细胞）之间的相互作用外，通过构成B细胞受体的表面免疫球蛋白传递的信号和一些遗传改变在CLL的发病过程中起作用。慢性淋巴细胞白血病的临床进展是异质性的，从诊断后不久需要治疗的患者到多年不需要治疗的患者，如果根本不需要治疗。在确定最佳治疗策略时，应考虑几个因素，包括免疫球蛋白重链可变区基因（IGHV）突变状态、基因组变化、患者年龄和合并症的存在，这些因素包括化疗、化疗免疫治疗和/或靶向B细胞受体信号或凋亡抑制剂（如BCL-2）的药物。对CLL生物学的研究极大地增强了我们识别疾病进展高风险患者的能力，以及我们使用选择性靶向CLL独特表型或生理特征的药物治疗患者的能力。这些和其他进展如何塑造了我们目前对CLL患者的理解和治疗，这是本入门的主题。
Chronic lymphocytic leukaemia (CLL) is a malignancy of CD5+ B cells that is characterized by the accumulation of small, mature-appearing lymphocytes in the blood, marrow and lymphoid tissues. Signalling via surface immunoglobulin, which constitutes the major part of the B cell receptor, and several genetic alterations play a part in CLL pathogenesis, in addition to interactions between CLL cells and other cell types, such as stromal cells, T cells and nurse-like cells in the lymph nodes. The clinical progression of CLL is heterogeneous and ranges from patients who require treatment soon after diagnosis to others who do not require therapy for many years, if at all. Several factors, including the immunoglobulin heavy-chain variable region gene (IGHV) mutational status, genomic changes, patient age and the presence of comorbidities, should be considered when defining the optimal management strategies, which include chemotherapy, chemoimmunotherapy and/or drugs targeting B cell receptor signalling or inhibitors of apoptosis, such as BCL-2. Research on the biology of CLL has profoundly enhanced our ability to identify patients who are at higher risk for disease progression and our capacity to treat patients with drugs that selectively target distinctive phenotypic or physiological features of CLL. How these and other advances have shaped our current understanding and treatment of patients with CLL is the subject of this Primer.