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<p>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.</p>

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Hyperhemolysis in a sickle cell disease patient in pregnancy

  • 期刊论文

  • 出版模式：

    开放获取

  • 发表日期：

    2025年7月25日

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  • Kristen Warncke,
  • Alecia Nero,
  • Ravindra Sarode,
  • Brian D. Adkins,
  • Jamie Morgan,
  • Natalie Bavli

摘要

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.