原发性血小板增多症:一个表型谱与真性红细胞增多症-一个病例报告

T. Akingbola, O. Aworanti
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引用次数: 0

摘要

原发性血小板血症(ET)是一种非白血病骨髓增生性肿瘤(MPN),伴有巨核细胞显著增殖和血小板计数升高(≥450,000/mm3)。这种疾病的特点是脾肿大、血管并发症、血栓形成和/或出血性临床病程发作。尽管ET是一种独特的临床实体,但其表现出与其他非白血病性mpn(包括真性红细胞增多症(PV)和原发性骨髓纤维化(PMF))相似的特征表型谱,其特征是涉及类似的遗传驱动因素。在这个报告中,我们提出了一个病例的原发性血小板血症的妇女以前诊断为子宫肌瘤和顽固性血栓并发症。我们还强调了治疗性静脉切开术作为治疗血栓事件的重要方法的相关性。关键词:原发性血小板增多症,真性红细胞增多症,骨髓增生性肿瘤,血栓形成,血小板计数
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Essential thrombocythaemia: a phenotypic spectrum with polycythaemia vera – a case report
Essential thrombocythaemia (ET) is a non-leukaemic myeloproliferative neoplasm (MPN) associated marked proliferation of the megakaryocytes and elevated platelet count (≥450,000/mm3). This condition is characterized by splenomegaly, vascular complications, thrombotic and / or hemorrhagic clinical course episode. ET despite being a distinct clinical entity presents with characteristic phenotypic spectrum similar to the other non-leukemic MPNs including polycythaemia vera (PV) and primary myelofibrosis (PMF) and are characterized by the involvement of similar genetic drivers. In this report, we present a case of essential thrombocythaemia in a woman previously diagnosed with uterine fibroid and an intractable thrombotic complication. We also highlight the relevance of therapeutic phlebotomy as an important approach for management of thrombotic events.Keywords: Essential thrombocythaemia, Polycythaemia vera, myeloproliferative neoplasm, thrombosis, platelet count
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