Spontaneous Escherichia coli meningitis associated with hemophagocytic lymphohistiocytosis

Spontaneous Escherichia coli meningitis has not been previously reported in association with hemophagocytic lymphohistiocytosis (HLH). A previously healthy 72-year-old woman was admitted due to fever, nuchal rigidity, disturbed consciousness and splenomegaly. Anemia, thrombocytopenia and hyperferritinemia developed on the 8(th) day of hospitalization. Cultures of cerebrospinal fluid and blood grew E. coli. Abundant macrophages overwhelmed erythrocytes in the bone marrow aspirate, confirming the presence of hemophagocytosis. E. coli meningitis was managed with a 40-day course of antibiotic treatment. However, the severity of anemia and thrombocytopenia progressed despite intensive transfusion therapy. The patient died of HLH on the 60(th) day of hospitalization.