IVIG successfully halts a recurrent hyperhaemolysis syndrome in a young patient with sickle cell crisis

Abstract Hyperhaemolysis syndrome is a rare blood transfusion complication putting patients requiring repeat transfusions frequently at risk for this life-threatening condition. The mechanism is not completely understood but an immune reaction with complement and macrophage activation has been proposed. Splenic sequestration and aplastic crisis might be involved as well. We present a case of a 27-year-old male with a past medical history of sickle cell disease, hemochromatosis from blood transfusions, a previous episode of hyperhaemolysis syndrome, unprovoked pulmonary embolism, and aortic stenosis. The patient presented to the emergency department “ED” with lower back and right lower leg pain unrelieved with home pain meds, similar to his pain crisis, with hydrocodone providing temporary relief. Over the last year, he had 8 ED visits and 5 admissions for pain management and blood transfusions. On physical exam, the patient was hypotensive 99/67 and had tenderness in his right shin and lumbar spine with normal imaging. He also had a grade 3/5 systolic murmur in the right upper sternal border, which had been consistent with his aortic stenosis. His hemoglobin dropped from 8 to 5.6 mg/dl and the patient was transfused 2 units of packed red blood cells with methylprednisolone 40 mg IV prior, his hemoglobin dropped quickly afterward, reticulocytes spiked from 11% to 27%, Ferritin increased to 33K, further transfusions resulted in worse response. Hematology recommended stopping transfusions, starting IVIG 1g/kg for 5 days, and continuing steroids. On day 3, his hemoglobin started to increase after it reached an extreme low of 4.8 mg/dl, the patient was discharged after his hemoglobin was 7.3 mg/dl. Managing patients with recurrent hyperhaemolysis syndrome can be extremely stressful due to limited treatment data. Blood transfusions are not an option as they may precipitate further hemolysis. Currently only 5 known cases have been reported. The patient's significant response to IVIG along with methylprednisolone offers a valuable treatment option to guide physicians' decisions in the future. Initiating treatment with the biologic Tocilizumab is also an option which has shown benefit in similar cases.