Etoposide in patients with rheuma-associated hemophagocytic lymphohistiocytosis / macrophage activation syndrome

Background/aim Rheuma-associated hemophagocytic lymphohistiocytosis (Rh-HLH), also called macrophage activation syndrome (MAS), is a severe complication of systemic inflammatory disorders. Rh-HLH has clinical and laboratory similarities to other forms of HLH, and is potentially life threatening. Treatment of Rh-HLH has not been standardized yet, but it commonly includes a variety of agents such as high-dose corticosteroids, cyclosporine, intravenous immunoglobulin and, in severe cases, sometimes etoposide. Here we report on the experience of etoposide in two children with severe Rh-HLH admitted to the Karolinska Children’s Hospital over the 6 months July 2010 to Dec 2010.