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骨髓增殖性疾病继发下腔静脉血栓形成所致布加综合征

[Budd-Chiari syndrome secondary to thrombosis of the inferior vena cava in myeloproliferative disease].

作者信息

Foresti V, Ungaro A, Pediconi A M

机构信息

Divisione Medica III, U.S.S.L. 75/1, Milano, Presidio Multizonale Ospedaliero dell'E.R.S.Z.

出版信息

Minerva Med. 1988 Jun;79(6):477-80.

PMID:3380314
Abstract

A case with abdominal pain, hepatosplenomegaly and ascites is reported. Ultrasonography and computed tomography showed a thrombus localized in the inferior vena cava at the confluence of the hepatic veins. The diagnosis of Budd-Chiari syndrome was made two years after clinical and laboratory findings appeared. The patient was affected by a Philadelphia-positive myeloproliferative disease that was the predisposing condition to the inferior vena cava thrombosis. We suggest that the diagnosis of Budd-Chiari syndrome must be considered in patients presenting conditions associated with thrombotic diathesis: so a suitable therapy can be started before an irreversible hepatic damage.

摘要

报告了一例伴有腹痛、肝脾肿大和腹水的病例。超声检查和计算机断层扫描显示,在肝静脉汇合处的下腔静脉中有一个血栓。在临床和实验室检查结果出现两年后,确诊为布加综合征。患者患有费城染色体阳性的骨髓增殖性疾病,这是下腔静脉血栓形成的诱发因素。我们建议,对于存在与血栓形成素质相关病症的患者,必须考虑布加综合征的诊断:这样可以在发生不可逆的肝损伤之前开始适当的治疗。

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