Sharma Vipra, Khalid Aysha, Cohen Alice J
Department of Hematology Oncology, Newark Beth Israel Medical Center, Newark, New Jersey.
AJP Rep. 2013 May;3(1):29-32. doi: 10.1055/s-0032-1331376. Epub 2012 Dec 31.
Hemophilia type A is a rare inherited bleeding disorder with a diversity of clinical manifestations ranging from persistent bleeding after minor trauma, spontaneous deep muscle or joint hemorrhage, to intracranial hemorrhage. As an X-linked disorder, hemophilia is rare in females and therefore there is little experience with pregnancy and no standardized guidelines to prevent bleeding antepartum, at delivery, and postpartum. We report the clinical course and management of a woman with severe hemophilia A who on two occasions had uncomplicated pregnancies and vaginal deliveries at term utilizing bolus recombinant factor VIII concentrate.
甲型血友病是一种罕见的遗传性出血性疾病，临床表现多样，从轻微创伤后持续出血、自发性深部肌肉或关节出血到颅内出血。作为一种X连锁疾病，血友病在女性中罕见，因此在妊娠方面经验很少，也没有预防产前、分娩时和产后出血的标准化指南。我们报告了一名患有严重甲型血友病的女性的临床病程及治疗情况，该患者两次足月妊娠均顺利，并使用大剂量重组凝血因子VIII浓缩物经阴道分娩。
