Hemophagocytic Syndrome Complicating Cardiac Transplantation

Infection-associated hemophagocytic syndrome is one of the hemophagocytic disorders, and is most often seen in the pediatric population, typically in the setting of immunosuppression. We present the case of a 33-year-old man who had been well for more than 3 years following cardiac transplantation until he developed the infection-associated hemophagocytic syndrome. The patient had a fulminant downhill course, dying in shock 10 weeks after his first presentation. Serologic studies for Epstein-Barr virus suggested a remote infection; other viral and microbiologic studies were negative. The only previous report of infection-associated hemophagocytic syndrome complicating cardiac transplant appears to be that of a pediatric patient. The case presented illustrates the difficulties in antemortem diagnosis of this disorder, and in its treatment.