Hämophagozytische Lymphohistiozytose bei unspezifischer Virusinfektion

Lachmann, Gunnar; Knaak, Cornelia; La Rosée, Paul; Spies, Claudia; Nyvlt, Peter; Oberender, Christian; Sander, Leif Erik; Suttorp, Norbert; Müller-Redetzky, Holger

Hemophagocytic lymphohistiocytosis (HLH), also known as hemophagocytic syndrome or macrophage activation syndrome within a pre-existing rheumatological disease, remains undiagnosed in over 70% of all cases in intensive care units (ICU) due to the sepsis-like clinical presentation. This report describes the case of a 30-year-old previously healthy male patient who was admitted to the normal infectiology ward of the Charité – Universitätsmedizin Berlin with unclear fever after a 3‑month journey around Asian and South America. The patient was transferred to the ICU after 3 days because of respiratory failure. Due to the immediate diagnostics of HLH and initiation of specific immunosuppressive treatment with dexamethasone, immunoglobulins and anakinra, the patient completely recovered and could finally be discharged after a 2‑week stay in hospital. Furthermore, the current diagnostic and therapeutic options are discussed. Ferritin is a decisive diagnostic marker that should be determined in every patient with unclear organ failure.