Seheult Jansen N, Chibisov Irina
Department of Pathology, University of Pittsburgh School of Medicine, Pittsburgh, PA,
Department of Pathology, University of Pittsburgh School of Medicine, Pittsburgh, PA, Institute for Transfusion Medicine, University of Pittsburgh Medical Center Health System, Pittsburgh, PA.
Lab Med. 2016 Aug;47(3):233-40. doi: 10.1093/labmed/lmw023. Epub 2016 Jun 10.
Herein, we present the case of a 22-year old obese Caucasian woman female with no acquired thrombophilic risk factors who was diagnosed with extensive cerebral sinus thrombosis. A detailed thrombophilia workup demonstrated persistently elevated plasminogen activator inhibitor 1 (PAI-1) activity levels, with an elevated PAI-1 antigen concentration and homozygosity for the PAI-1 4G allele (4G/4G genotype). The patient was treated with indefinite warfarin anticoagulation medication due to the unprovoked nature of her thrombotic event. Disturbances in the fibrinolytic system, in particular PAI-1, have been related to an increased risk of arterial and venous thrombosis. In this article, we discuss the pathophysiology of hypofibrinolysis associated with elevated PAI-1 levels and the PAI-1 4G/5G polymorphism.
在此,我们报告一例22岁的肥胖白种女性病例,该患者无获得性血栓形成风险因素,却被诊断为广泛脑静脉窦血栓形成。详细的血栓形成倾向检查显示纤溶酶原激活物抑制剂1(PAI-1)活性水平持续升高,PAI-1抗原浓度升高,且PAI-1 4G等位基因纯合(4G/4G基因型)。由于其血栓形成事件为自发性,该患者接受了长期华法林抗凝治疗。纤溶系统紊乱,尤其是PAI-1,与动脉和静脉血栓形成风险增加有关。在本文中,我们讨论了与PAI-1水平升高和PAI-1 4G/5G多态性相关的低纤溶血症的病理生理学。