Hyperhemolysis syndrome in anemia of chronic disease

Occasional cases of delayed hemolytic transfusion reaction (DHTR) demonstrate severe and persistent hemolysis and are referred to as hyperhemolysis syndrome. This syndrome usually occurs in patients with sickle cell disease and possibly thalassemia who receive multiple transfusions. There are few such clinical reports in patients without hemoglobinopathies.

A 67-year-old woman with anemia and a history of four previous transfusions was admitted with shortness of breath and a hematocrit (Hct) level of 27 percent. The patient was group O with a negative antibody screen. She received 1 unit of electronically cross-matched red blood cells (RBCs) and was discharged. Thirteen days later she returned to hospital with weakness and a Hct level of 23 percent. The antibody screen now demonstrated anti-K alloantibody. The direct antigloblulin test (DAT) was positive with both anti-immunoglobulin G and anti-complement (C3). Anti-K was recovered in the eluate. The previously transfused RBC unit was positive for presence of the K antigen. The patient's RBCs were negative for the presence of K antigen. Other laboratory data confirmed ongoing hemolysis, and a diagnosis of DHTR was made. She continued to display findings of active hemolysis for 9 more weeks requiring 19 units of RBCs. Thirty-four days after the original transfusion, her DAT remained positive and both the plasma sample and a RBC eluate demonstrated anti-K.

The delayed hemolytic transfusion reaction with hyperhemolysis can occur among patients without hemoglobinopathies.