Qadir Hira, Rashid Anila, Adil Salman Naseem
Section of Hematology, Department of Pathology and Laboratory Medicine, The Aga Khan University Hospital, Karachi.
J Coll Physicians Surg Pak. 2017 Sep;27(9):S86-S88.
Factor VII (FVII) deficiency is one of the rare inherited bleeding disorders. Thrombosis has been occasionally described in inherited FVII deficiency. Here, we report a young female with undiagnosed FVII deficiency who presented with cerebral venous sinus thrombosis (CVST). Oral contraceptive pill was found to be prothrombotic risk factor. The CVSToccurred in spite of the congenital FVII deficiency indicating that no definitive antithrombotic protection is assured by this defect. Low molecular weight heparin and anti-Xa assay were found to be safe choice of anticoagulation and monitoring, respectively, in this patient.
凝血因子VII（FVII）缺乏症是一种罕见的遗传性出血性疾病。遗传性FVII缺乏症中偶尔会出现血栓形成的情况。在此，我们报告一名年轻女性，她患有未确诊的FVII缺乏症，表现为脑静脉窦血栓形成（CVST）。发现口服避孕药是血栓形成的危险因素。尽管存在先天性FVII缺乏症，但仍发生了CVST，这表明这种缺陷并不能确保绝对的抗血栓保护作用。在该患者中，发现低分子量肝素和抗Xa测定分别是抗凝和监测的安全选择。
