Royal Marsden NHS Foundation Trust, London, UK.
Institute of Cancer Research, London, UK.
Curr Oncol Rep. 2024 Feb;26(2):129-135. doi: 10.1007/s11912-023-01485-3. Epub 2024 Jan 2.
This review summarises the recent advances in knowledge regarding the biology and treatment of prolymphocytic leukaemias.
Both B-PLL and T-PLL are genetically complex, and the molecular landscape of these diseases has been well characterised recently. Diagnostic criteria for T-PLL have been refined with the publication of the first international consensus criteria, whereas the diagnosis of B-PLL has been thrown into question by the most recent WHO classification. Treatment advances in B-PLL have relied heavily on the advances seen in CLL that have then been extrapolated to B-PLL with just a few case reports to support the use of these targeted inhibitors. Despite increased knowledge of the biology of T-PLL and some elegant pre-clinical models to identify potential treatments, unfortunately, no improvements have been made in the treatment of T-PLL. Unmet need is a term oft used for many diseases, but this is particularly true for patients with prolymphocytic leukaemias. Ongoing improvements in our understanding of these diseases will hopefully lead to improved therapies in the future.
本文总结了近年来关于幼淋巴细胞白血病生物学和治疗的最新进展。
B-PLL 和 T-PLL 在遗传上均较为复杂，且这些疾病的分子特征最近已得到很好的描述。随着首个国际共识标准的发布，T-PLL 的诊断标准已得到完善，而 B-PLL 的诊断标准则因最近的世界卫生组织分类而受到质疑。B-PLL 的治疗进展在很大程度上依赖于 CLL 的治疗进展，然后将这些靶向抑制剂外推至 B-PLL，仅有少数病例报告支持这些抑制剂的使用。尽管对 T-PLL 的生物学有了更多的了解，并建立了一些优雅的临床前模型来确定潜在的治疗方法，但 T-PLL 的治疗仍未得到改善。未满足的需求是许多疾病的常用术语，但对于幼淋巴细胞白血病患者来说尤其如此。随着我们对这些疾病的理解不断提高，希望未来能有更好的治疗方法。
