Sorrentino Francesco, Maffei Laura, Caprari Patrizia, Cassetta Rita, Dell'Anna Donatella, Materazzi Stefano, Risoluti Roberta
Thalassemia Unit, S. Eugenio Hospital, Rome, Italy.
National Centre for the Control and Evaluation of Medicines, Istituto Superiore di Sanità, Rome, Italy.
Front Mol Biosci. 2020 Feb 14;7:16. doi: 10.3389/fmolb.2020.00016. eCollection 2020.
The life expectancy of thalassemia patients has increased significantly in recent years being the most "elderly" patients approaching or are over 50 years old. Consequently, patients' perspectives have changed, leading them to longer-term planning with a consequent increase in their reproductive potential and desire to have children. Crucial points in the management of pregnancy in thalassemia are the iron chelation therapy before and during pregnancy, the antithrombotic prophylaxis, the management of transfusion therapy according to the modified transfusion requirement, a cardiologic monitoring for hemodynamic changes that expose an increased risk of heart failure. Pregnancy in women with sickle cell disease is still associated with increased rates of maternal and fetal mortality and adverse outcomes. Maternal morbidity may be due to acute sickling crises, thromboembolism, infection, and chronic end-organ dysfunction, while neonatal outcomes may be intrauterine growth retardation, preterm delivery, small infants for gestational age, stillbirth, and neonatal death. The management of pregnancy in thalassemia and sickle cell disease requires to be approached by a multidisciplinary team and followed from the pre-conception phase until the post-partum period with a close monitoring of the maternal and fetal conditions, in order to ensure optimal outcome. This approach requires the application of well-defined protocols that cover all the critical aspects of pregnancies in women affected by these pathologies. We describe our experience of spontaneous and non-spontaneous pregnancies in patients with thalassemia major and intermedia and sickle cell disease followed between 1992 and 2018 at the Thalassemia Unit of S. Eugenio Hospital of Rome.
近年来，地中海贫血患者的预期寿命显著增加，最年长的患者已接近或超过50岁。因此，患者的观念发生了变化，促使他们进行更长期的规划，从而增加了生殖潜力和生育意愿。地中海贫血患者孕期管理的关键点包括孕前和孕期的铁螯合治疗、抗血栓预防、根据调整后的输血需求进行输血治疗管理，以及针对暴露于心力衰竭风险增加的血流动力学变化进行心脏监测。镰状细胞病女性怀孕仍与母婴死亡率增加及不良结局相关。孕产妇发病可能归因于急性镰状细胞危象、血栓栓塞、感染和慢性终末器官功能障碍，而新生儿结局可能是宫内生长受限、早产、小于胎龄儿、死产和新生儿死亡。地中海贫血和镰状细胞病患者的孕期管理需要多学科团队参与，从孕前阶段到产后阶段都要密切监测母婴状况，以确保最佳结局。这种方法需要应用涵盖这些疾病女性妊娠所有关键方面的明确方案。我们描述了1992年至2018年期间在罗马圣欧金尼奥医院地中海贫血科对重型和中间型地中海贫血及镰状细胞病患者的自然妊娠和非自然妊娠的治疗经验。
