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一名年轻原发性血小板增多症女性患者的大型主动脉血栓的药物治疗

Medical management of a large aortic thrombus in a young woman with essential thrombocythemia.

作者信息

Fang M, Agha S, Lockridge L, Lee R, Cleary J P, Mazur E M

机构信息

Department of Medicine, Norwalk Hospital, Yale University School of Medicine, Conn, USA.

出版信息

Mayo Clin Proc. 2001 Apr;76(4):427-31. doi: 10.4065/76.4.427.

Abstract

Aortic thrombus formation is rare in the patients with essential thrombocytosis (ET); therefore, no guidelines for its management have been established. Embolism from ET-associated large vessel thrombi is potentially lethal and has been managed surgically in a few reported cases. We describe herein a 45-year-old black woman with ET found to have a 3.5-cm, pedunculated intra-aortic thrombus at the thoracoabdominal junction. How to treat this potentially devastating aortic thrombus was a management dilemma. We believed, based on the patient's diagnosis of ET and the histology of similar thrombi in 1 reported series, that the aortic thrombus was a "white thrombus" consisting primarily of aggregated platelets with a minimal fibrin network and almost no entrapped erythrocytes. The patient was treated with aspirin, 325 mg daily, as a platelet antiaggregating agent and hydroxyurea, 1,500 mg daily, to reduce the platelet count to less than 450 x 10(9)/L. The thrombus resolved without severe thromboembolic events. To our knowledge, this is the first reported case of a large intra-aortic thrombosis associated with ET that has been successfully managed with medical therapy alone.

摘要

原发性血小板增多症(ET)患者中主动脉血栓形成较为罕见;因此,尚未制定关于其治疗的指南。ET相关的大血管血栓形成的栓塞具有潜在致死性,在少数已报道的病例中采用了手术治疗。我们在此描述一名45岁的黑人女性ET患者,在胸腹交界处发现有一个3.5厘米的带蒂主动脉内血栓。如何治疗这个潜在的毁灭性主动脉血栓是一个治疗难题。基于患者的ET诊断以及1个报道系列中类似血栓的组织学情况,我们认为该主动脉血栓是一个“白色血栓”,主要由聚集的血小板组成,纤维蛋白网络极少,几乎没有包埋的红细胞。患者接受了每日325毫克阿司匹林作为血小板抗聚集剂治疗,以及每日1500毫克羟基脲治疗,以使血小板计数降至低于450×10⁹/L。血栓消退,未发生严重血栓栓塞事件。据我们所知,这是首例报道的与ET相关的主动脉内大血栓形成仅通过药物治疗成功治愈的病例。

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