Hemophagocytic syndrome

This case report is about an elderly man who presented with a long-standing history of high-grade fever and weight loss. He initially had only hepatos plenomegaly, but then developed jaundice. He also had pancytopenia and rais ed liver enzymes. His septic screen was negative, but he had a positive Mon ospot test and immunoglobulin G for Epstein-Barr virus. The liver biopsy sh owed sinusoidal phagocytosis and the subsequent bone marrow aspiration and biopsy showed significant hemophagocytosis, hence Hemophagocytic syndrome w as diagnosed. The fever was refractory to antibiotic and anti-tuberculosis therapy, but it responded only partially to steroids. Full response was onl y noticed following antiviral treatment in the form of intravenous Ganciclo vir. The patient's general condition, liver enzymes, bilirubin, hematologic al parameters and even the weight returned back to their normal range 2 wee ks after Ganciclovir therapy. Cessation of this drug resulted in relapse of his symptoms and oral antivirals did not help. Splenectomy, steroid pulse therapy and immunosuppressive treatment were only partially helpful. Reintr oduction of Ganciclovir did help for a short period. We conclude that our p atient had virus-associated hemophagocytic syndrome most likely related to Epstein-Barr virus infection, which was then confirmed by the splenic biops y, and that Ganciclovir can be of great help in eradicating the virus and t reating the disease, provided that it is given for a long enough period.