Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, MN 55905, USA.
Leuk Res. 2012 Dec;36(12):1481-9. doi: 10.1016/j.leukres.2012.08.011. Epub 2012 Aug 21.
Polycythemia vera, essential thrombocythemia and primary myelofibrosis constitute the BCR-ABL1-negative myeloproliferative neoplasms. In this communication, I will provide an overview on their histopathology, cytogenetic findings and associated mutations, as well as summarize recent advances that have changed our approach to their diagnosis and treatment. Also included in the current review are (i) indications for ordering JAK2 or MPL mutation analysis and result interpretation, (ii) new international prognostic scoring systems, and (iii) risk-adapted therapy including the therapeutic role of immunomodulatory drugs and JAK inhibitors.
真性红细胞增多症、原发性血小板增多症和原发性骨髓纤维化构成了 BCR-ABL1 阴性骨髓增殖性肿瘤。在本次交流中,我将概述其组织病理学、细胞遗传学发现和相关突变,并总结改变我们对其诊断和治疗方法的最新进展。本次综述还包括:(i) 申请 JAK2 或 MPL 突变分析的指征及结果解读;(ii) 新的国际预后评分系统;以及 (iii) 风险适应性治疗,包括免疫调节药物和 JAK 抑制剂的治疗作用。