Basophilia Predicts Poorer Outcomes in Essential Thrombocythemia, Polycythemia Vera, Primary Myelofibrosis, and Myeloproliferative Neoplasm, Unclassifiable

IF 10.1 1区 医学 Q1 HEMATOLOGY American Journal of Hematology Pub Date : 2024-11-22 DOI:10.1002/ajh.27530
Lisa Yuen, Tasos Gogakos, Leonardo Boiocchi, Gabriela Hobbs, Robert Hasserjian
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Abstract

Basophils are hematopoietic cells derived from myeloid progenitor cells and are found in increased numbers in some myeloid neoplasms, particularly chronic myeloid leukemia (CML). Marked basophilia is a poor prognostic indicator in CML and defines accelerated phase according to the revised 4th edition World Health Organization (WHO4R) classification and the International Consensus Classification (ICC).

Basophilia is less well-documented in the classic BCR::ABL1-negative myeloproliferative neoplasms (MPNs) essential thrombocythemia (ET), polycythemia vera (PV), and primary myelofibrosis (PMF). The clinical behavior of these BCR::ABL1-negative MPNs is heterogeneous, with varying propensity to progress to fibrotic or blast phase. Prior studies have reported that patients with primary or secondary myelofibrosis (MF) with high basophil counts have increased risk of progression to blast phase, shortened survival, and more frequent CALR mutations [1].

The significance of basophilia across ET, PV, and PMF, as well as its association with driver mutations other than the classic JAK2 V617F, CALR, and MPL, have not been previously characterized. In the current study, we examined a broad cohort of MPNs to determine associations of basophilia with clinical and molecular features and patient outcome.

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嗜碱性粒细胞增多症预示着重要血小板增多症、多发性红细胞瘤、原发性骨髓纤维化和骨髓增生性肿瘤(不可分类)的预后较差
嗜碱性粒细胞是源自髓系祖细胞的造血细胞,在一些髓系肿瘤,尤其是慢性髓性白血病(CML)中,嗜碱性粒细胞的数量会增加。根据世界卫生组织(WHO)修订的第四版(WHO4R)分类和国际共识分类(ICC),明显的嗜碱性粒细胞增多是 CML 的不良预后指标,也是加速期的定义。这些BCR::ABL1阴性MPN的临床表现各不相同,进展到纤维化期或坏死期的倾向也各不相同。先前的研究报告称,嗜碱性粒细胞计数高的原发性或继发性骨髓纤维化(MF)患者进展到鼓风期的风险增加,生存期缩短,CALR突变更频繁[1]。嗜碱性粒细胞增多在ET、PV和PMF中的意义,及其与经典的JAK2 V617F、CALR和MPL以外的驱动突变的关联,先前尚未定性。在目前的研究中,我们研究了一大批多发性骨髓瘤患者,以确定嗜碱性粒细胞增多与临床和分子特征及患者预后的关系。
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来源期刊
CiteScore
15.70
自引率
3.90%
发文量
363
审稿时长
3-6 weeks
期刊介绍: The American Journal of Hematology offers extensive coverage of experimental and clinical aspects of blood diseases in humans and animal models. The journal publishes original contributions in both non-malignant and malignant hematological diseases, encompassing clinical and basic studies in areas such as hemostasis, thrombosis, immunology, blood banking, and stem cell biology. Clinical translational reports highlighting innovative therapeutic approaches for the diagnosis and treatment of hematological diseases are actively encouraged.The American Journal of Hematology features regular original laboratory and clinical research articles, brief research reports, critical reviews, images in hematology, as well as letters and correspondence.
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