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骨髓增殖性肿瘤

Myeloproliferative Neoplasms

作者信息

Thapa Bicky, Fazal Salman, Parsi Meghana, Rogers Heesun J.

机构信息

Cleveland Clinic

Allegheny Health Network

Abstract

Hematopoietic pluripotent stem cells have self-renewal capability and give rise to either myeloid or the lymphoid lineage which further differentiates into various mature blood cells such as red blood cells (RBC), lymphocytes, granulocytes, megakaryocytes, and macrophages. The hematopoietic process is determined by the bone marrow environment, growth factors, and transcription factors. The abnormal proliferation of one or more terminal myeloid cell lines in the peripheral blood gives rise to a heterogeneous group of disorders called myeloproliferative neoplasms. In 1951, William Dameshek coined the term which are now reformed to myeloproliferative neoplasms (MPNs) by the World Health Organization (WHO). Chronic myeloid leukemia (CML), polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF) are four classic types of myeloproliferative neoplasms. WHO classification also included chronic neutrophilic leukemia (CNL), chronic eosinophilic leukemia (CEL), and MPN, unclassifiable. Out of the classic types of MPNs, CML is positive, but PV, ET, and PMF are negative. Besides, the fourth edition of WHO classification for myeloid and acute leukemia was revised in 2016 due to recent advances in hematology with the identification of molecular markers and prognostic markers, giving a better understanding of the molecular pathogenesis and genetics of the hematological malignancies.

摘要

造血多能干细胞具有自我更新能力,可分化为髓系或淋巴系,进而进一步分化为各种成熟血细胞,如红细胞(RBC)、淋巴细胞、粒细胞、巨核细胞和巨噬细胞。造血过程由骨髓环境、生长因子和转录因子决定。外周血中一种或多种终末髓系细胞系的异常增殖会引发一组异质性疾病,称为骨髓增殖性肿瘤。1951年,威廉·达梅谢克创造了这个术语,现在世界卫生组织(WHO)将其重新命名为骨髓增殖性肿瘤(MPN)。慢性粒细胞白血病(CML)、真性红细胞增多症(PV)、原发性血小板增多症(ET)和原发性骨髓纤维化(PMF)是骨髓增殖性肿瘤的四种经典类型。WHO分类还包括慢性中性粒细胞白血病(CNL)、慢性嗜酸性粒细胞白血病(CEL)和无法分类的MPN。在MPN的经典类型中,CML呈阳性,而PV、ET和PMF呈阴性。此外,由于血液学的最新进展,包括分子标志物和预后标志物的鉴定,WHO髓系和急性白血病分类的第四版于2016年进行了修订,从而更好地理解血液系统恶性肿瘤的分子发病机制和遗传学。

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