Department of Haematology, University of Cambridge, Cambridge, United Kingdom.
Hematology Am Soc Hematol Educ Program. 2009:621-8. doi: 10.1182/asheducation-2009.1.621.
The last four years have seen an explosion in our understanding of the myeloproliferative neoplasms. Important and often unexpected insights into the molecular mechanisms responsible for these disorders have been accompanied by the development of new diagnostic tests and by an improved understanding of the relationship between the different disease entities. This review will focus on recent developments in the pathogenesis and management of essential thrombocythemia with a particular emphasis on its phenotypic overlap with polycythemia vera and primary myelofibrosis.
过去四年中,我们对骨髓增殖性肿瘤的认识有了爆发式的增长。对这些疾病的分子机制的重要认识,而且经常是出乎意料的认识,伴随着新的诊断测试的发展和对不同疾病实体之间关系的理解的提高。这篇综述将重点关注原发性血小板增多症发病机制和治疗方面的最新进展,特别强调其与真性红细胞增多症和原发性骨髓纤维化的表型重叠。