Résumé

Background: Red blood cell (RBC) transfusion is the cornerstone of therapy for sickle cell patients and significantly reduces the morbidity and mortality attributable to sickle cell disease. However, transfusion is not without complications such as iron overload, infections, and especially delayed post-transfusion hemolysis, which may jeopardize the vital prognosis of the sickle cell patient. Objective: This study aims to document and analyze a series of four cases of delayed post-transfusion hemolysis, diagnosed and managed at the National Reference Center for Sickle Cell Disease (CNRD) in Niamey, Niger. Clinical case: This concerns four patients who presented with delayed post-transfusion hemolysis. Corticosteroid therapy based on methylprednisolone at 2 mg/kg/day for three days was instituted. A blood transfusion was performed after the first dose of corticosteroid therapy. The patients were placed on hydroxyurea one month after their hospitalization. Results: The outcome was favorable for all cases, with progressive regression of respiratory signs, clinical signs, and biological signs of hemolysis. Initiation of hydroxyurea prevented any new transfusion and vaso-occlusive crisis. Hemoglobin levels remained consistently above 5.5 g/dL. Conclusion: Corticosteroid therapy based on methylprednisolone at 2 mg/kg/day for three days appears to have been beneficial in the management of delayed post-transfusion hemolysis in sickle cell patients.

Mot-clés :

Sickle Cell Disease, Hemolysis, Post-Transfusion, Corticosteroid Therapy, Niger