背景:Glanzmann血栓形成(GT)是一种罕见的,血小板糖蛋白IIb-IIIa受体的常染色体隐性遗传疾病。妊娠GT患者母体和胎儿出血的风险增加。关于患者围产期管理的文献很少。
方法:我们介绍了通过多学科方法管理的GT患者的产前至产后过程,包括跨母胎医学的沟通,血液学,输血医学,和麻醉服务。除了常规产前产科影像学和血液学实验室研究,我们每4~6周主动监测患者的抗血小板抗体,以评估新生儿同种免疫性血小板减少症的风险.此外,我们优先考虑子宫内膜,氨甲环酸,和输血HLA匹配的血小板,以控制产后期间母亲和胎儿的出血。
结论:迄今为止,在GT患者的妊娠期间管理出血或预防同种免疫方面的指南有限.这里,我们介绍了一个复杂的病例,对母亲进行预防性出血积极管理,同时对母亲和胎儿进行围产期出血风险和事件的连续监测.此外,未来的研究需要继续评估这些方法,以减轻后续妊娠中出血风险的增加.
Glanzmann thrombasthenia (GT) is a rare, autosomal recessive disorder of platelet glycoprotein IIb-IIIa receptors. Pregnant patients with GT are at increased risk of maternal and fetal bleeding. There is a paucity of literature on the peripartum management of patients.
We present the antepartum through the postpartum course of a patient with GT who was managed by a multidisciplinary approach that included communication across maternal-fetal medicine, hematology, transfusion medicine, and anesthesiology services. In addition to routine prepartum obstetric imaging and hematologic laboratory studies, we proactively monitored the patient for anti-platelet antibodies every 4-6 weeks to gauge the risk for neonatal alloimmune thrombocytopenia. Furthermore, we prioritized uterotonics, tranexamic acid, and transfusion of HLA-matched platelets to manage bleeding for mother and fetus intrapartum through the postpartum periods.
To date, there are limited guidelines for managing bleeding or preventing
alloimmunization during pregnancy in patients with GT. Here, we present a complex
case with aggressive management of bleeding prophylactically for the mother while serially monitoring both mother and fetus for peripartum bleeding risks and events. Moreover, future studies warrant continued evaluation of these approaches to mitigate increased bleeding risks in subsequent pregnancies.