Ruxolitinib as first-line therapy in secondary hemophagocytic lymphohistiocytosis and HIV infection.

Secondary lymphohistiocytosis is a severe inflammatory condition characterized by uncontrolled inflammatory response hyperactivation of antigen-presenting cells, hemophagocytosis, cytopenias, and multiorgan failure, and is secondary to subjacent pathologies such as cancer, infection, or immune disease. Standard treatment includes chemotherapy; however, this is not always possible or safe. Ruxolitinib, a Janus kinase (JAK) 1/2 inhibitor can reduce inflammation and cytokine activity and has been used in refractory cases and first-line treatment. Here, we present a 35-year-old patient with HIV infection and secondary lymphohistiocytosis due to CMV/EBV infection who was successfully treated with ruxolitinib as first-line therapy. He rapidly showed clinical and biochemical improvement and transfusion independence without complications.