早期或纤维化前原发性骨髓纤维化与原发性血小板增多症鉴别的研究进展  

Prefibrotic primary myelofibrosis versus essential thrombocythemia: clinical, laboratory, morphological, inflammatory status, molecular, prognostic and therapeutic comparisons

作　　者：白雪 赵一帆 冯志金 苏永忠[1] 陶红芳[1] BAI Xue;ZHAO Yifan;FENG Zhijin

机构地区：[1]汕头大学医学院第一附属医院血液内科,广东汕头515041

出　　处：《临床血液学杂志》2021年第3期220-224,共5页Journal of Clinical Hematology

摘　　要：骨髓增殖性肿瘤(myeloproliferative neoplasms,MPN)是起源于异常克隆性增殖的造血干细胞、以髓系一系或多系过度增殖为主要特征的一组疾病。原发性血小板增多症(essential thrombocythemia,ET)与原发性骨髓纤维化(primary myelofibrosis,PMF)同属于BCR-ABL融合基因阴性的MPN。The 2016 revision of the World Health Organization(WHO) classification of myeloproliferative neoplasms defines 2 stages of primary myelofibrosis(PMF): the prefibrotic/early(pre-PMF) phase and the overt fibrotic(overt PMF) phase. Pre-PMF minimicks essential thrombocythemia(ET) in clinical presentation and routine laboratory examination, but shows higher rates of progression to overt-PMF, leukemic evolution and shorter overall survival comparing with ET. Thus, it is critical to distinguish pre-PMF from ET for both prognostic implications and therapeutic decisions. The current review provides an overview on how to diagnose and treat pre-PMF comparing with ET, according to clinical and laboratory parameters, bone marrow morphological changes, inflammatory status and gene mutation, with the aim of preventing the progress of the disease and improving the prognosis.

关 键 词：早期或纤维化前原发性骨髓纤维化 原发性血小板增多症 核因子E2 慢性炎症 巨核细胞 

分 类 号：R551.3[医药卫生—血液循环系统疾病]