The Royal Marsden Hospital and the Institute of Cancer Research, Biomedical Research Centre, Sutton, UK.
Br J Haematol. 2020 Nov;191(4):527-534. doi: 10.1111/bjh.17158.
For a disease initially described in 1958 as a leukaemic reticulo-endotheliosis associated with poor outcomes, we have come a long way in our understanding of Hairy cell leukaemia. The vast majority of patients diagnosed with this rare, often diagnostically challenging, leukaemia can now expect a lifespan that is similar to the general population. This article covers some of the highlights from the last 6 decades that have led to our current understanding of this fascinating leukaemia - from elucidation of its B-cell origin to discovery of the almost universal occurrence of the BRAF V600E mutation; from the initial successes reported with splenectomy to the more recent development of targeted therapies such as Vemurafenib and Moxetumomab Pasudotox. It also pays tribute to some of the outstanding research in this field focusing particularly on the significant contributions made by the clinical and scientific community in the UK.
对于一种最初于 1958 年被描述为与不良预后相关的白血病网状内皮细胞增多症的疾病，我们在对毛细胞白血病的理解上已经取得了长足的进步。现在，绝大多数被诊断患有这种罕见的、通常具有诊断挑战性的白血病的患者，可以预期其寿命与普通人群相似。本文涵盖了过去 60 年来的一些重要进展，这些进展使我们对这种引人入胜的白血病有了目前的认识——从阐明其 B 细胞起源到发现几乎普遍存在的 BRAF V600E 突变；从最初报道的脾切除术的成功，到最近开发的靶向治疗方法，如vemurafenib 和 moxetumomab pasudotox。它还向该领域的一些杰出研究致敬，特别关注英国临床和科学界的重大贡献。
