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原发性血小板增多症及其并发症的综述。

A review of essential thrombocythemia and its complications.

机构信息

Department of Leukemia, The University of Texas MD Anderson Cancer Center, Houston, Texas.

出版信息

Clin Adv Hematol Oncol. 2023 Feb;21(2):76-84.

Abstract

Essential thrombocythemia (ET) is a chronic myeloproliferative neoplasm characterized by an increased platelet count in the peripheral blood and excessive megakaryopoiesis in the bone marrow. In one-third of cases, the disease remains benign and does not lead to complications. In the remaining cases, however, ET may present with thromboembolic and hemorrhagic complications and transform into more aggressive myeloid neoplasms, with a negative effect on morbidity and mortality. Despite extensive research and a better understanding of the pathogenesis and etiology of the complications associated with ET, limited data are available on the management of complications and emergencies. This article highlights the complications and emergencies associated with ET and discusses treatment options and the controversies associated with management.

摘要

原发性骨髓纤维化(ET)是一种慢性骨髓增殖性肿瘤,其特征是外周血血小板计数增加,骨髓中巨核细胞过度生成。在三分之一的病例中,该疾病保持良性,不会导致并发症。然而,在其余病例中,ET 可能出现血栓栓塞和出血并发症,并转化为更具侵袭性的髓系肿瘤,对发病率和死亡率产生负面影响。尽管对 ET 相关并发症的发病机制和病因进行了广泛的研究和更好的理解,但关于并发症和急症的管理的数据有限。本文重点介绍了 ET 相关并发症和急症,并讨论了治疗选择和管理相关的争议。

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