The Walter and Eliza Hall Institute of Medical Research, Parkville, Victoria, Australia.
Leuk Lymphoma. 2013 May;54(5):922-33. doi: 10.3109/10428194.2012.734615. Epub 2012 Oct 24.
The understanding of myeloproliferative neoplasms has changed dramatically since Dameshek proposed his classification over 50 years ago. Our knowledge of the types of cells which constitute the hematopoietic system and of how they are regulated has also appreciated significantly over this time. This review relates what is currently known about the acquired genetic mutations associated with adult myeloproliferative neoplasms to how they lead to the hematopoietic perturbations of myeloproliferative disease. There is a particular focus on how stem and progenitor cell compartments are affected by BCR-ABL1 and JAK2V617F mutations, and the particular issue of resistance of leukemic stem cells to conventional and targeted therapies.
自 Dameshek 提出他的分类 50 多年以来，人们对骨髓增生性肿瘤的认识已经发生了巨大的变化。在此期间，我们对构成造血系统的细胞类型及其调节方式的了解也有了显著的提高。本综述介绍了目前已知的与成人骨髓增生性肿瘤相关的获得性遗传突变及其如何导致骨髓增生性疾病的造血紊乱。特别关注 BCR-ABL1 和 JAK2V617F 突变如何影响干细胞和祖细胞区室，以及白血病干细胞对常规和靶向治疗的耐药性这一特殊问题。
