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

白雪; 赵一帆; 冯志金; 苏永忠; 陶红芳

doi:10.13201/j.issn.1004-2806.2021.03.016

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

- 汕头大学医学院第一附属医院血液内科(广东汕头, 515041)

详细信息

通讯作者: 陶红芳,E-mail:taohongfangchn@163.com

中图分类号: R551.3

收稿日期: 2020-05-14

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

More Information

Corresponding author: TAO Hongfang, E-mail: taohongfangchn@163.com

Received Date: 14 May 2020

摘要

- 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
Abstract: 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.
- early/prefibrotic primary myelofibrosis /
- essential thrombocythemia /
- NF-E2 /
- chronic inflammation /
- megakaryocyte

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参考文献(47)

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