文章摘要

原发性骨髓纤维化的临床与分子生物学预后因素的研究进展

作者: 1杜金环, 1赵艳红, 1周晋
1 哈尔滨医科大学附属第一医院血液内科,哈尔滨 150001
通讯: 周晋 Email: zhoujin1111@126.com
DOI: 10.3978/j.issn.2095-6959.2019.03.033
基金: 黑龙江自然科学基金 (D201252)。

摘要

原发性骨髓纤维化( pr imar y myelof i brosi s,PMF)是典型的费城染色体阴性骨髓增生性肿瘤(myeloproliferative neoplasm,MPN)中最具侵袭性的一种克隆性疾病,临床表现包括脾肿大、全血细胞减少和一系列可能使人衰弱的全身症状。其病程可变,中位生存期从几个月到几年。临床和生物学特征如高龄,炎症标志物升高和铁稳态失衡等可影响PMF患者的预后。TET2,IDH1/2和ASXL1突变等新的分子标志物也被确定为预后变量。本文就PMF中最新发现的临床、生物学、分子遗传学等预后因素及治疗进展进行综述。
关键词: 原发性骨髓纤维化;基因突变;预后

Research progress in clinical and molecular biological prognostic factors of primary myelofibrosis

Authors: 1DU Jinhuan, 1ZHAO Yanhong, 1ZHOU Jin
1 Department of Hematology, First Affiliated Hospital of Harbin Medical University, Harbin 150001, China

CorrespondingAuthor: ZHOU Jin Email: zhoujin1111@126.com

DOI: 10.3978/j.issn.2095-6959.2019.03.033

Foundation: This work was supported by the Natural Science Foundation of Heilongjiang Province, China (D201252).

Abstract

Primary myelofibrosis is one of the most aggressive clonal diseases in a typical Philadelphia chromosome-negative myeloproliferative neoplasm. The course of the disease is variable and the median survival time ranges from a few months to a few years. Clinical and biological characteristics such as aging, elevated inflammatory markers and imbalance of iron homeostasis can affect the prognosis of patients with primary myelofibrosis. New molecular markers such as TET2, IDH1/2 and ASXL1 mutations have also been identified as prognostic variables. This article reviews the latest clinical, biological, molecular genetic prognostic factors and treatment progress in primary myelofibrosis.
Keywords: primary myelofibrosis; gene mutations; prognosis