The use of cyclosporin-A in the treatment of virus-associated hemophagocytic syndrome in adults.

Virus-associated hemophagocytic syndrome (VAHS) is one of the categories of hemophagocytic syndrome (HPS) which is a clinicopathologic entity characterized by systemic proliferation of benign hemophagocytic cells of the monocyte-macrophage-histiocyte lineage, associated with fever, cytopenia, hepatosplenomegaly, lymphadenopathy, and coagulopathy. The disease affects children and adults after infection with various viruses. However, clinical manifestations are quite different between children and adults. Although the etiology of VAHS is not fully understood, accumulating evidence has revealed dysregulation of the immune system. Standard treatment of VAHS has not been established, yet, but successful trials aiming at immunomodulation have been reported. In this report, current concepts and clinical features of VAHS are reviewed, focusing on the recently introduced cyclosporin A therapy in VAHS in adults.