Gauthier M
Service d'hématologie-maladies du sang, centre hospitaler de Cahors, Cahors, France.
Rev Med Interne. 2022 Jun;43(6):356-364. doi: 10.1016/j.revmed.2022.01.010. Epub 2022 Feb 17.
Chronic lymphocytic leukemia (CLL) is a low-grade B cell lymphoma with circulating cells, often revealed by hyperlymphocytosis. Its diagnosis and therapeutic indications (not systematic) have been defined in 2018. In fact, CLL can be separated in two entities differentiating themselves by their IGHV mutational status, but the search of other prognostic parameters like TP53 disruption is mandatory before treatment. Numerous genetic alterations and mutations exist in CLL. CLL cells are highly dependent from their b-cell receptor stimulation and from their microenvironment, which takes a central place in disease progression. Infections, dysimmune manifestations, cancers and Richter transformation are classic complications, and patients have poor vaccine response even without a treatment. Chemoimmunotherapy is being challenged by the new highly active drugs such as Bruton tyrosine-kinase inhibitors (ibrutinib, acalabrutinib) and by the association of venetoclax and anti-CD20. Future treatment strategies might integrate both new drugs and classical chemoimmunotherapy.
慢性淋巴细胞白血病（CLL）是一种伴有循环细胞的低度B细胞淋巴瘤，常因淋巴细胞增多而被发现。其诊断和治疗指征（并非系统性的）已于2018年确定。事实上，CLL可分为两个实体，它们通过IGHV突变状态相互区分，但在治疗前必须寻找其他预后参数，如TP53缺失情况。CLL存在众多基因改变和突变。CLL细胞高度依赖其B细胞受体刺激及其微环境，而微环境在疾病进展中起着核心作用。感染、免疫失调表现、癌症和Richter转化是典型并发症，即使未经治疗，患者的疫苗反应也较差。化学免疫疗法正受到新型高活性药物（如布鲁顿酪氨酸激酶抑制剂（伊布替尼、阿卡拉布替尼））以及维奈克拉与抗CD20联合用药的挑战。未来的治疗策略可能会整合新药和经典化学免疫疗法。
