Casas Patarroyo Claudia Patricia, Agudelo López Claudia Del Pilar, Galvez Kenny, Lagos Ibarra Jimmy, Martínez Rojas Susan, Ibatá Bernal Linda
Hospital San José, Fundación Universitaria de Ciencias de la Salud, Bogotá, Colombia.
Clínica Colsanitas, Organización Sanitas, Bogotá, Colombia.
Rev Med Chil. 2019 Mar;147(3):334-341. doi: 10.4067/S0034-98872019000300334.
Acquired hemophilia A (AHA) is a rare and life-threatening autoimmune hemorrhagic disorder where autoantibodies are developed against factor VIII. An early diagnosis is challenging and mandatory: an immediate hemostatic control is required to reduce morbidity and mortality. Laboratory features of AHA are: presence of autoantibodies against factor VIII, prolonged activated partial thromboplastin time (with normal prothrombin time and thrombin time) and decreased factor VIII levels. In some cases, the results of laboratory tests may be incorrect due to errors in analysis, blood extraction or manipulation of samples; also worth of consideration are limitations in the measurement range and low sensitivity of the tests. This review highlights the importance of adequate screening in patients with suspected AHA to make an adequate diagnosis and reduce overall fatal outcomes.
获得性血友病A(AHA)是一种罕见且危及生命的自身免疫性出血性疾病,患者体内会产生针对凝血因子VIII的自身抗体。早期诊断具有挑战性且至关重要:需要立即进行止血控制以降低发病率和死亡率。AHA的实验室特征包括:存在针对凝血因子VIII的自身抗体、活化部分凝血活酶时间延长(凝血酶原时间和凝血酶时间正常)以及凝血因子VIII水平降低。在某些情况下,由于分析、采血或样本处理错误,实验室检测结果可能不准确;检测的测量范围限制和低灵敏度也值得考虑。本综述强调了对疑似AHA患者进行充分筛查以做出准确诊断并降低总体致命后果的重要性。