Ann Hematol. 2025 Jul 25. doi: 10.1007/s00277-025-06264-7. Online ahead of print.
ABSTRACT
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.
PMID:40707630 | DOI:10.1007/s00277-025-06264-7
