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Hyperhemolysis in sickle cell disease

Elena Aragona1, Michael J Kelly

  • 1*Division of Hospitalist Medicine, Children's National Medical Center, The George Washington University School of Medicine and Health Sciences, Washington, DC †Division of Pediatric Hematology Oncology, The Floating Hospital for Children at Tufts Medical Center, Tufts University School of Medicine, Boston, MA.

Journal of Pediatric Hematology/Oncology
|April 27, 2013
PubMed

An 18-year-old female with sickle cell disease presented with thigh pain, dark urine, and hematuria within 72 hours of receiving a blood transfusion. Her clinical picture was consistent with hemolysis. Subsequent laboratory workup, however, demonstrated reticulocytopenia without evidence of an antibody-mediated transfusion reaction. As her hemoglobin continued to decrease, she was treated with IVIG and steroids for presumed hyperhemolysis. Clinicians should have a high index of suspicion for hyperhemolysis in sickle cell patients with evidence of hemolysis after a recent transfusion. Differentiating hyperhemolysis from other hemolytic syndromes is critical; transfusions in a hyperhemolytic episode can accelerate hemolysis causing life-threatening anemia.

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