Warncke Kristen, Nero Alecia, Sarode Ravindra, Adkins Brian D, Morgan Jamie, Bavli Natalie
Department of Obstetrics & Gynecology, University of Texas Southwestern Medical Center, Dallas, TX, USA.
Department of Obstetrics and Gynecology, University of Texas Southwestern Medical Center, 5323 Harry Hines Boulevard, Dallas, TX, 75390-9032, USA.
Ann Hematol. 2025 Jul 25. doi: 10.1007/s00277-025-06264-7.
Sickle cell disease (SCD) is the most commonly inherited hemoglobinopathy with the potential for significant maternal and perinatal morbidity and mortality. Hyperhemolysis syndrome (HS) is a rare, and potentially fatal complication of blood transfusion characterized by destruction of both transfused and autologous red blood cells, often resulting in severe anemia with post-transfusion hemoglobin levels lower than pre-transfusion levels. Evidence-based guidance on the prevention and management of HS during pregnancy is limited. We report the case of a 23-year-old gravida 1 para 0 with sickle cell anemia and a history of two prior episodes of HS, successfully managed through a multidisciplinary approach. Her care team included hematologists, maternal-fetal medicine specialists, transfusion medicine pathologists, and neonatologists. Management during pregnancy involved initiating hydroxyurea in the second trimester, implementing a transfusion-restricted protocol, and utilizing immunosuppression and blood conservation strategies. This case highlights the successful application of a multidisciplinary approach to the management of a high-risk pregnancy complicated by SCD and recurrent HS. Key interventions included the use of hydroxyurea, erythropoiesis-stimulating agents, immunosuppression, and a planned delivery strategy, resulting in a favorable maternal and neonatal outcome.
镰状细胞病（SCD）是最常见的遗传性血红蛋白病，可能导致严重的孕产妇和围产期发病及死亡。高溶血综合征（HS）是一种罕见且可能致命的输血并发症，其特征是输入的和自身的红细胞均被破坏，常导致严重贫血，输血后血红蛋白水平低于输血前水平。关于孕期HS预防和管理的循证指南有限。我们报告了一例23岁初孕0产的镰状细胞贫血患者，有两次HS发作史，通过多学科方法成功管理。她的护理团队包括血液科医生、母胎医学专家、输血医学病理学家和新生儿科医生。孕期管理包括在孕中期开始使用羟基脲，实施输血限制方案，以及采用免疫抑制和血液保护策略。该病例突出了多学科方法在管理合并SCD和复发性HS的高危妊娠中的成功应用。关键干预措施包括使用羟基脲、促红细胞生成剂、免疫抑制和计划性分娩策略，从而取得了良好的母婴结局。
