How I treat essential thrombocythemia.

How I treat essential thrombocythemia.
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DOI:
10.1182/blood-2010-08-270033
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发表时间:
2011-02-03
期刊:
影响因子:
20.3
通讯作者:
Green AR
Green AR
中科院分区:
医学1区
文献类型:
--
作者:
Beer PA;Erber WN;Campbell PJ;Green AR

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在过去的5年里,我们已经目睹了显着的进步,在诊断过程和最佳治疗原发性血小板增多症(ET)患者。对潜在分子机制的深入了解伴随着新的诊断测试的发展,以及对ET与其他相关骨髓增生性肿瘤(如真性红细胞增多症和原发性骨髓纤维化)之间关系的更好理解。在这篇综述的第一部分中,我们描述了如何将最近的分子和组织学研究整合到一个适用于日常临床实践的简化诊断过程中。我们还讨论了目前诊断争议的领域,包括ET内的异质性和ET、真性红细胞增多症和原发性骨髓纤维化之间的表型重叠。在第二部分,我们提供了一个概述,我们目前的方法来治疗ET,包括危险分层,选择细胞减灭剂,并考虑特殊情况下,如怀孕或围手术期的病人。讨论的争议领域包括确定那些在高风险的并发症和治疗决策的年轻患者。
In the past 5 years we have witnessed significant advances in both the diagnostic process and optimal therapy for patients with essential thrombocythemia (ET). Insights into the underlying molecular mechanisms have been accompanied by the development of new diagnostic tests and by an improved understanding of the relationship between ET and other related myeloproliferative neoplasms, such as polycythemia vera and primary myelofibrosis. In the first part of this review, we describe how recent molecular and histologic studies can be integrated into a streamlined diagnostic process that is applicable to everyday clinical practice. We also address areas of current diagnostic controversy, including heterogeneity within ET and the phenotypic overlap between ET, polycythemia vera, and primary myelofibrosis. In the second part, we provide an overview of our current approach to the treatment of ET, including risk stratification, choice of cytoreductive agent, and a consideration of special situations such as the pregnant or perioperative patient. Areas of controversy discussed include the identification of those at high risk of complications and therapeutic decisions in the younger patient.
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