摘要
原发性骨髓纤维化(PMF)是骨髓增殖性肿瘤(MPN)的一种,在MPN中预后最差。免疫调节剂、促红细胞生成素、细胞毒性药物、脾切除术等可以显著减轻患者症状,但并不能改善患者预后,异基因造血干细胞移植仍然是唯一可能治愈PMF的方法。已经上市的JAK激酶抑制剂芦可替尼及正在研究中的去甲基化药物、抗纤维化药物、端粒酶抑制剂等为PMF的治疗提供了新的思路,使逆转骨髓纤维化的自然病程成为可能。文章将对PMF的治疗研究进展进行综述。
Primary myelofibrosis (PMF) is a type of myeloproliferative neoplasms (MPN) with the worst prognosis in MPN. Immunomodulators, erythropoiesis-stimulating agents, cytoreductive drugs, splenectomy, etc. can significantly reduce the symptoms of patients, but can not improve the prognosis of patients. Allogeneic hematopoietic stem cell transplantation remains the only method that may cure PMF. The marketed JAK kinase inhibitor ruxolitinib and the hypomethylating agents, anti-fibrosis agents and telomerase inhibitor provide new ideas for the treatment of PMF, made it possible to reverse the natural course of myelofibrosis. This paper reviews the progress of PMF therapy.
作者
宁海玲
张秀莲
Ning Hailing;Zhang Xiulian(Department of Hematology,the First Hospital of Shanxi Medical University,Taiyuan 030001,China)
出处
《白血病.淋巴瘤》
CAS
2018年第10期628-631,共4页
Journal of Leukemia & Lymphoma