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Sex-Related Differences in Chronic Myeloid Neoplasms: From the Clinical Observation to the Underlying Biology

机译:慢性骨髓肿瘤的性与相关差异:从临床观察到潜在生物学

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摘要

Chronic myeloid neoplasms are clonal diseases with variable clinical course and outcomes and despite the introduction of novel therapies, patients with high-risk disease continue to have overall poor outcomes. Different groups have highlighted that men have overall worse survival and higher incidence of transformation to acute leukemia compared to women across neoplasms such as myelodysplastic syndrome (MDS), myeloproliferative neoplasms (MPN), MDS/MPN overlap neoplasms, and CML. More recent studies evaluating the genomic profile of patients with these neoplasms demonstrated a male predominance for mutations in high-risk genes including ASXL1, U2AF1, SRSF2 and ZRSR2. The understanding of the underlying biology is limited but a number of hypotheses have been developed and are currently being investigated. This review summarizes the current knowledge about sex-related differences in the clinical outcomes and genomic profile of patients with chronic myeloid neoplasms and discusses the hypothesized biologic mechanisms as an attempt to explain these observations.
机译:慢性骨髓肿瘤是具有可变临床课程和结果的克隆疾病,尽管引入了新的疗法,高危疾病的患者仍然存在较差的结果。与骨髓增生综合征(MDS),Myeloproiferative型肿瘤(MPN),MDS / MPN),MDS / MPN重叠肿瘤和CML等肿瘤中的女性相比,不同的团体突出了男性对急性白血病的转化总体活跃和转化发病率更高。最近的研究评估了这些肿瘤的患者的基因组剖面表明,在包括ASXL1,U2AF1,SRSF2和ZRSR2中的高风险基因中的突变中展示了男性优势。对潜在生物学的理解是有限的,但已经开发了许多假设,目前正在调查。本综述总结了关于慢性髓鞘肿瘤患者的临床结果和基因组概况的当前关于与慢性骨髓肿瘤患者的基因组概况的知识,并讨论了假设的生物机制,以试图解释这些观察。

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