HISTORY UND CLINICAL FINDINGS: Within a few weeks, two patients aged 16 and 75 years with fever of unknown origin were referred to the emergency unit.
Laboratory tests revealed increased aminotransferase levels, progressive cytopenia and an increase of inflammatory markers. In the older patient a rapid clinical detoriation with multi organ failure was observed. Progressively increasing levels of ferritin and soluble-interleukin-2-receptor levels led to the diagnosis of hemophagocytic lymphohistiocytosis, which was confirmed by bone marrow examination in the case of the younger patient.
Immunsuppressive treatment induced a clear improvement of clinical and laboratory findings and in the case of the older patient finally led to convalescence.
Hemophagocytic lymphohistiocytosis is a rare but potentially fatal differential diagnosis, which should be considered in patients with fever and cytopenia.