Problem diagnostyczny: gorÄ czkujÄ cy nastolatek z rzadkim zespołem chorobowym

Haemophagocytic syndrome (hemophagocytic lymphohistiocytosis, HLH) is a rare hematologic disorder which manifests itself by inflammatory reaction caused by incorrect activation of immune cells. A 16-year-old boy was admitted to the hospital with a high fever, respiratory distress, dyspnea, rash and hepatosplenomegaly. The differential diagnosis initially considered infectious mononucleosis and Kawasaki disease. Eventually the boy was transferred to the Department of Pediatric Oncology, Hematology and Transplantology in Poznań in which, in the course of extensive diagnosis, HLH triggered by HHV-6 infection, was established. Treatment was initiated according to the HLH 2004 protocol. Clinical improvement was achieved.