Hyperhemolysis Syndrome in a Pediatric Patient with Sickle Cell Disease

Hyperhemolysis syndrome is a rare delayed post-transfusion hemolytic reaction, characterized by the destruction of transfused and autologous red blood cells, that occurs mostly in patients with chronic hemolysis. In patients with sickle cell disease severe complications may develop, with an associated significant morbidity and mortality. We describe the case of a 17-year-old female patient with sickle cell disease with poor transfusional yield, associated with a high hemoglobin S percentage (92%) and significant hemolysis, that lead to a diagnosis of hyperhemolysis syndrome. A multidisciplinary management approach included limiting red blood cell transfusions, supportive care with darbepoetin, therapeutic plasma exchange, intravenous immunoglobulin, and steroids, which resulted in a favorable clinical response. This case aims to shed light on a rare entity to avoid additional transfusions, which can further exacerbate hemolysis, and to ensure an appropriate management of this potentially fatal condition.