López Miguel, Fica Matías, Soto Verónica, Samaniego Francisco
Hospital San José, Santiago, Chile.
Centro de Referencia Nacional Diagnóstico de Hemofilia, Hospital Roberto del Río, Santiago, Chile.
Rev Med Chil. 2024 Jan;152(1):119-123. doi: 10.4067/s0034-98872024000100119.
Tissue Factor-Factor VII complex is essential in coagulation activation. Congenital factor VII deficiency is a rare disorder that has an autosomal recessive inheritance. Clinical presentations are heterogeneous, ranging from asymptomatic carriers to severe bleeding phenotypes with factor VII replacement therapy requirements. Treatments options are plasma derived and recombinants FVII concentrates or fresh frozen plasma in case that first options are not available. In pregnancy factor VII levels increase in women with mild and moderate deficiencies but not in severe deficiency. The management of pregnant women with factor FVII deficiency must be done by a multidisciplinary team of hematologist, obstetrics and anesthetist and should be guided by the women bleeding history, the coagulations test, levels of factor FVII and rout of delivery. We present the case of a 31-year-old pregnant woman who, due to an alteration in prothrombin time, is diagnosed with Factor VII deficiency and its respective obstetric management.
组织因子 - 因子VII复合物在凝血激活中至关重要。先天性因子VII缺乏是一种罕见的疾病，呈常染色体隐性遗传。临床表现具有异质性，从无症状携带者到需要因子VII替代治疗的严重出血表型不等。治疗选择包括血浆源性和重组FVII浓缩物，若无法获得首选方案则使用新鲜冷冻血浆。在孕期，轻度和中度缺乏的女性因子VII水平会升高，但重度缺乏者不会。患有因子FVII缺乏的孕妇必须由血液科医生、产科医生和麻醉师组成的多学科团队进行管理，并应以女性的出血史、凝血试验、因子FVII水平和分娩途径为指导。我们报告一例31岁孕妇的病例，该孕妇因凝血酶原时间改变被诊断为因子VII缺乏及其相应的产科处理情况。
