Hyperhemolysis in a sickle cell disease patient in pregnancy

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.