Department of Haematology, University Hospital Southampton. UK.
Clin Med (Lond). 2013 Apr;13(2):188-92. doi: 10.7861/clinmedicine.13-2-188.
The myeloproliferative neoplasms that are associated with the JAK2 mutation are a heterogeneous group of disorders. The additional mutations that result in the clinical phenotype are still the subject of research. As more than one mutation is involved, and as JAK2 has a necessary physiological role (unlike BCR-ABL), the development of targeted therapy remains a challenge. Although new drugs are being developed, treatment at present is predominantly with agents that have been in use for many years. An understanding of the need to control the thrombotic risk has, however, led to improved survival rates such that ET and PV can be seen as chronic diseases.
与 JAK2 突变相关的骨髓增殖性肿瘤是一组异质性疾病。导致临床表型的其他突变仍在研究中。由于涉及多种突变，并且 JAK2 具有必要的生理作用（与 BCR-ABL 不同），因此靶向治疗的开发仍然是一个挑战。尽管正在开发新的药物，但目前的治疗主要是使用多年来一直在使用的药物。然而，对控制血栓形成风险的认识导致了生存率的提高，使得 ET 和 PV 可以被视为慢性疾病。
