妊娠期和围产期轻度血友病和血友病携带者的管理和结局:美国血友病治疗中心的经验

Hana I Lim, M. Desancho
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引用次数: 1

摘要

背景:在美国,轻度血友病患者和血友病携带者的妊娠、围产期管理和结局尚未得到很好的确定。目的了解我院血友病治疗中心(HTC)轻度血友病患者及血友病携带者在辅助受孕、妊娠、围生期及产后的处理及结局。方法回顾性分析2008年1月至2020年10月在我院随访的轻度血友病A或B孕妇(因子VIII [FVIII]或因子IX [FIX]水平<0.4 IU/mL)和血友病A、B携带者的电子病历。统计数据、诊断原因、基线和妊娠晚期FVIII和FIX水平、出血表型和基因型。记录妊娠方式、因子替代、补铁、分娩方式、麻醉方式、围生期并发症及子代结局。结果12例妊娠18例,其中轻度a型血友病2例,轻度B型血友病2例,a型血友病携带者6例,B型血友病携带者2例。11例妊娠(61%)为自然妊娠,7例(39%)为体外受精妊娠。经阴道分娩8例(44.4%),剖腹产10例(55.6%)。17例(94.4%)分娩采用轴向麻醉,无并发症。4例妊娠(22.2%)出现出血并发症,其中2例为不需要输血的产后出血。在我们的病例系列中,妊娠血友病携带者和轻度血友病患者,通过仔细详细的多学科驱动方法取得了成功的结果。
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Management and outcomes of mild hemophiliacs and hemophilia carriers during pregnancy and peripartum period: a hemophilia treatment center experience in the United States
Abstract Background Pregnancy, peripartum management, and outcomes of mild hemophiliacs and hemophilia carriers in the United States are not well established. Aim To describe the management and outcomes of mild hemophiliacs and hemophilia carriers during assisted conception, pregnancy, peripartum and post-partum period at our hemophilia treatment center (HTC). Methods Retrospective review of electronic medical records of pregnant women with mild hemophilia A or B (Factor VIII [FVIII] or Factor IX [FIX] level <0.4 IU/mL) and hemophilia A and B carriers followed at our HTC from January 2008 to October 2020. Demographics, the reason for diagnosis, FVIII and FIX levels at baseline and third trimester, bleeding phenotype and genotype were obtained. Method of conception, factor replacement, iron supplementation, mode of delivery, type of anesthesia, peripartum complications, and offspring outcomes was recorded. Results There was a total of 18 pregnancies in 12 women (2 with mild hemophilia A, 2 mild hemophilia B, 6 hemophilia A carriers, and 2 hemophilia B carriers). Eleven pregnancies (61%) were conceived naturally and 7 (39%) via in-vitro fertilization (IVF). Eight (44.4%) and 10 (55.6%) pregnancies were vaginal and C-section deliveries, respectively. Neuraxial anesthesia was administered in 17 (94.4%) deliveries without complications. Four pregnancies (22.2%) had bleeding complications, 2 of which were post-partum hemorrhages not requiring transfusion. Conclusion In our case series of pregnant hemophilia carriers and mild hemophiliacs, successful outcomes were achieved with a carefully detailed multidisciplinary-driven approach.
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