Setaka Tamao, Hirano Katsuharu, Moriya Keiichi, Kaneko Tougen, Morita Seie, Shinkai Tetsu, Morishita Eriko, Ichida Takafumi
Department of Internal Medicine, Shonan East General Hospital, Japan.
Department of Clinical Laboratory Sciences, Graduate School of Medical Science, Kanazawa University, Japan.
Intern Med. 2019 Jun 15;58(12):1733-1737. doi: 10.2169/internalmedicine.2295-18. Epub 2019 Feb 25.
Portal vein thrombosis (PVT) has been reported in many patients with and without liver cirrhosis. The portal vein is a rare site of thrombosis, and various conditions can predispose an individual to PVT. Among those conditions, hereditary thrombophilia has been increasingly reported recently. We herein report the case of a non-cirrhotic 30-year-old man who developed acute PVT with hereditary antithrombin deficiency. Antithrombin (AT) replacement therapy was required along with heparin. Given our experience with this case, we believe that a screening test for prothrombotic disorders, such as AT deficiency, should be considered in cases of PVT.
门静脉血栓形成（PVT）在许多肝硬化患者和非肝硬化患者中均有报道。门静脉是血栓形成的罕见部位，多种情况可使个体易患PVT。在这些情况中，遗传性血栓形成倾向最近报道得越来越多。我们在此报告一例30岁非肝硬化男性患者，其因遗传性抗凝血酶缺乏而发生急性PVT。除肝素外，还需要进行抗凝血酶（AT）替代治疗。鉴于我们处理该病例的经验，我们认为对于PVT病例，应考虑对诸如AT缺乏等血栓形成前疾病进行筛查。
