Research progress of pathogenesis in polycythemia vera

Lixia Fu, Yingshao Wang
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Abstract

Polycythemia vera (PV) is representative in Ph- myeloproliferative neoplasm (MPN), which is characterized by increasing of two or three line blood cells, especially erythrocytosis. First, during the occurrence and progression of PV, JAK2, TET2, IDH1/2 and ASXL1 gene mutation and associated epigenetic changes play an important role. Besides, abnormal cytokines produced by chronic inflammation can promote the malignant transformation of hematopoietic stem cells (HSC), which may also be one of the important mechanisms of PV occurrence. Moreover, the changes in hematopoietic microenvironment were also associated with the progression of post-PV myelofibrosis(MF). This article reviews the pathogenesis of PV from several aspects that involve genes mutation, chronic inflammation and the changing of hematopoietic microenvironment. Key words: Polycythemia vera; Hematopoietic stem cells; Mutation; Cytokines; Tumor microenvironment
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真性红细胞增多症发病机制的研究进展
真性红细胞增多症(PV)在骨髓增生性肿瘤(MPN)中具有代表性,其特征是两三线血细胞增多,尤其是红细胞增多。首先,在PV的发生和发展过程中,JAK2、TET2、IDH1/2和ASXL1基因突变和相关的表观遗传学变化起着重要作用。此外,慢性炎症产生的异常细胞因子可促进造血干细胞(HSC)的恶性转化,这可能也是PV发生的重要机制之一。此外,造血微环境的变化也与PV后骨髓纤维化(MF)的进展有关。本文从基因突变、慢性炎症和造血微环境变化等方面综述了PV的发病机制。关键词:真性红细胞增多症;造血干细胞;突变;细胞因子;肿瘤微环境
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期刊介绍: The International Journal of Transfusion and Hematology was founded in September 1978. It is a comprehensive academic journal in the field of transfusion and hematology, supervised by the National Health Commission and co-sponsored by the Chinese Medical Association, West China Second Hospital of Sichuan University, and the Institute of Transfusion Medicine of the Chinese Academy of Medical Sciences. The journal is a comprehensive academic journal that combines the basic and clinical aspects of transfusion and hematology and is publicly distributed at home and abroad. The International Journal of Transfusion and Hematology mainly reports on the basic and clinical scientific research results and progress in the field of transfusion and hematology, new experiences, new methods, and new technologies in clinical diagnosis and treatment, introduces domestic and foreign research trends, conducts academic exchanges, and promotes the development of basic and clinical research in the field of transfusion and hematology.
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