Hyperhemolysis Syndrome Following Red Cell Exchange in a Newly Diagnosed Sickle Cell Disease Patient With Spinal Cord Infarction

David Starr, Daniela Hermelin, Douglas Blackall


Hyperhemolysis syndrome (HS) is a rare red blood cell (RBC) transfusion reaction that shares similarities with other hemolytic transfusion reactions. Because of this, it is important to recognize key presenting clinical and laboratory features in order to guide therapy. In this case report, a patient with a sickling hemoglobinopathy who developed HS is presented. The atypical nature of this case resides in the clinical presentation of paraplegia secondary to spinal cord infarction, increasingly complex blood group serological findings, and multiple RBC exchanges prior to the HS reaction. Once the patient was diagnosed with probable HS, approximately 4 weeks into her clinical course, RBC transfusion (including exchange transfusion) was withheld. Instead, corticosteroids and erythropoietin were initiated without complication. The patient remained stable with this treatment modality until her care was transferred to a hospital with a comprehensive sickle cell center. This case highlights the need to withhold transfusion in HS patients, barring exceptional circumstances, and the efficacy of initiating immunomodulatory and erythropoiesis stimulating therapies.

J Med Cases. 2020;11(9):263-266
doi: https://doi.org/10.14740/jmc3541


Blood transfusion; Exchange transfusion; Hemolysis; Sickle cell disease; Sickling hemoglobinopathy; Transfusion reaction

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