Hyperhemolysis Syndrome in a Patient with Sickle Cell Disease: A Case Report.
INTRODUCTION: Hyperhemolysis syndrome (HHS) is a rare complication of repeat blood transfusions in sickle cell disease (SCD). This can occur acutely or have a delayed presentation and often goes unrecognized in the emergency department (ED) due to its rapid progression and similarity to acute chest syndrome and other common complications of SCD.
CASE REPORT: We present a case of a 20-year-old male with SCD who presented to the ED with pain and tenderness in his lower extremities one day after discharge for a crisis. Unbeknownst to the ED team, during his admission he had received a blood transfusion. On presentation he was noted to have hyperkalemia, hyperbilirubinemia, anemia, and uncontrolled pain, and was admitted for sickle cell pain crisis. Over the next 36 hours, his hemoglobin dropped precipitously from 8.9 grams per deciliter (g/dL) to 4.2 g/dL (reference range: 11.5-14.5 g/dL), reticulocyte count from 11.7 % to 3.8% (0.4-2.2%), and platelets from 318,000 per cubic centimeter (K/cm
CONCLUSION: Because of the untoward outcomes associated with delay in HHS diagnosis and the need for early initiation of steroids, it is important for emergency providers to screen patients with hemoglobinopathies for recent transfusion at ED presentation. Asking the simple question about when a patient's last transfusion occurred can lead an emergency physician to include HHS in the differential and work-up of patients with sickle cell disease complications.
Published In/Presented At
Kalter, J. A., Gupta, R., Greenberg, M. R., Miller, A. J., & Allen, J. (2021). Hyperhemolysis Syndrome in a Patient with Sickle Cell Disease: A Case Report. Clinical practice and cases in emergency medicine, 5(1), 101–104. https://doi.org/10.5811/cpcem.2020.12.50349
Emergency Medicine | Hematology | Medicine and Health Sciences
Department of Emergency Medicine, Department of Emergency Medicine Faculty, Department of Emergency Medicine Residents, Department of Medicine, Hematology-Medical Oncology Division, Fellows and Residents, USF-LVHN SELECT Program, USF-LVHN SELECT Program Faculty, USF-LVHN SELECT Program Students