Fiore Mathieu, Sentilhes Loïc, d'Oiron Roseline
Hematology Laboratory, Reference Center for Inherited Platelet Disorders, University Hospital of Bordeaux, Pessac, France.
Biology of Cardiovascular Diseases, INSERM U1034, Pessac, France.
Blood. 2022 Apr 28;139(17):2632-2641. doi: 10.1182/blood.2021011595.
Glanzmann thrombasthenia (GT) is a rare inherited platelet function disorder caused by a quantitative and/or qualitative defect of the αIIbβ3 integrin. Pregnancy and delivery are recognized risk periods for bleeding in women with GT. The newborn may also be affected by fetal and neonatal immune thrombocytopenia induced by the transplacental passage of maternal anti-αIIbβ3 antibodies, which can lead to severe hemorrhage and fetal loss. Pregnancy in women with GT thus requires a multidisciplinary approach, including prepregnancy counseling and a treatment plan for delivery for both the mother and child. In this article, we summarize the current knowledge on pregnancy in women with GT and describe how we manage this severe platelet disorder in our clinical practice.
血小板无力症（GT）是一种罕见的遗传性血小板功能障碍，由αIIbβ3整合素的数量和/或质量缺陷引起。妊娠和分娩是GT女性出血的公认危险期。新生儿也可能受到母体抗αIIbβ3抗体经胎盘传递所致的胎儿和新生儿免疫性血小板减少症的影响，这可能导致严重出血和胎儿丢失。因此，GT女性的妊娠需要多学科方法，包括孕前咨询以及针对母亲和孩子的分娩治疗计划。在本文中，我们总结了目前关于GT女性妊娠的知识，并描述了我们在临床实践中如何管理这种严重的血小板疾病。
