Barg Assaf A, Livnat Tami, Kenet Gili
The Israeli National Hemophilia Center and Thrombosis Institute, Sheba Medical Center, Tel Hashomer, Israel; Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
The Israeli National Hemophilia Center and Thrombosis Institute, Sheba Medical Center, Tel Hashomer, Israel; Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
Thromb Res. 2017 Mar;151 Suppl 1:S82-S85. doi: 10.1016/S0049-3848(17)30074-9.
Acquired hemophilia A (AHA) is a severe bleeding disorder caused by autoantibodies against clotting factor VIII (FVIII). With an estimated annual incidence of 1.3 to 1.5 per million, AHA is a rare disease. An extremely rare form of AHA has been described among women in the peripartum period, and may present with peripartum hemorrhage. Notably, although hemorrhagic symptoms commonly present 1-4 months around delivery, they may occur up to 1 year after parturition. When caring for a mother with AHA it is important to note that Factor VIII inhibitor may be transferred via the placenta from the mother to the fetus. Hence the newborn may also be affected. It is important to increase the awareness of Gynecologists for clinical symptoms and laboratory signs of AHA in order to avoid delayed diagnosis. Treatment may involve use of bypass agents to control hemorrhage, despite the risk of thrombosis, while immunomodulation (with increasing role for Rituximab) may be required to eradicate the inhibiting antibodies. Our review will evaluate the epidemiology, diagnosis, clinical course and treatment of peripartum AHA, focusing upon mother and infant care.
获得性血友病A（AHA）是一种由抗凝血因子VIII（FVIII）自身抗体引起的严重出血性疾病。AHA发病率估计为每年百万分之1.3至1.5，是一种罕见疾病。围产期女性中已描述了一种极其罕见的AHA形式，可能表现为围产期出血。值得注意的是，虽然出血症状通常在分娩前后1 - 4个月出现，但也可能在产后长达1年时发生。在护理患有AHA的母亲时，重要的是要注意VIII因子抑制剂可能通过胎盘从母亲转移到胎儿。因此新生儿也可能受到影响。提高妇科医生对AHA临床症状和实验室体征的认识很重要，以避免诊断延迟。治疗可能涉及使用旁路药物来控制出血，尽管有血栓形成风险，而可能需要进行免疫调节（利妥昔单抗的作用日益增加）以消除抑制性抗体。我们的综述将评估围产期AHA的流行病学、诊断、临床过程和治疗，重点关注母婴护理。
