hyperhemolysis syndrome
Pronunciation: hy-per-hee-MOL-ih-sis
A severe complication of blood transfusion in sickle cell disease where both transfused and native red blood cells are rapidly destroyed.
Full Definition
Hyperhemolysis syndrome is a life-threatening complication that can occur in patients with sickle cell disease following blood transfusion, characterized by destruction of both transfused donor cells and the patient's own red blood cells. This results in a post-transfusion hemoglobin level that may be lower than pre-transfusion values, along with evidence of hemolysis including elevated lactate dehydrogenase, indirect bilirubin, and reticulocytosis. The syndrome may be mediated by alloantibodies or complement activation and can lead to severe anemia and hemodynamic instability. Management is challenging and may require immunosuppression, plasma exchange, or supportive care while avoiding further transfusions if possible.
Usage
Usage note: Emphasize the paradoxical drop in hemoglobin post-transfusion as a key diagnostic feature.
In Context
- "The patient developed hyperhemolysis syndrome with a post-transfusion hemoglobin lower than baseline." — transfusion medicine consultation
- "Recognition of hyperhemolysis syndrome led to discontinuation of transfusions and initiation of immunosuppressive therapy." — hematology case conference