Chaari M, Sassi M, Galea V, Gerotziafas G T, Elalamy I
Service d'hématologie biologique, hôpital Tenon, Assistance publique-Hôpitaux de Paris, 4, rue de la Chine, 75020 Paris cedex 20, France.
Rev Med Interne. 2012 Jul;33(7):401-4. doi: 10.1016/j.revmed.2012.04.018. Epub 2012 May 30.
Acquired hemophilia A (AH) is a rare hemorrhagic disorder, secondary to the occurrence of factor VIII inhibitor. In young patients, this disorder is commonly observed during the post-partum period, and has been rarely documented in the prepartum. We report a new case of a prepartum AH and review literature data.
An isolated prolongation of the activated partial thromboplastin time (APTT) was fortuitously discovered in a 31-year-old pregnant women, with spontaneous ecchymosis of her lower limbs few days prior to delivery. Coagulation tests revealed decreased factor VIII activity (18%) and the presence of factor VIII inhibitor (1,4 Bethesda unit). In order to eradicate the autoantibody, the patient was first treated with prednisone and then with rituximab.
Prepartum factor VIII inhibitors need to be precociously recognized to allow prophylactic management of the delivery bleeding.
获得性血友病A（AH）是一种罕见的出血性疾病，继发于VIII因子抑制剂的出现。在年轻患者中，这种疾病常见于产后时期，而产前很少有记录。我们报告一例产前AH的新病例并回顾文献资料。
在一名31岁孕妇分娩前几天偶然发现活化部分凝血活酶时间（APTT）单独延长，伴有下肢自发性瘀斑。凝血试验显示VIII因子活性降低（18%）以及存在VIII因子抑制剂（1.4贝塞斯达单位）。为了消除自身抗体，患者首先接受泼尼松治疗，然后接受利妥昔单抗治疗。
产前VIII因子抑制剂需要尽早识别，以便对分娩出血进行预防性处理。
