#00061447

71 year-old man with hereditary hemochromatosis (heterozygous for H63D mutation in HFE gene) who presented for fever of unknown origin. Evaluation revealed disseminated histoplasmosis and clinical criteria was met for hemophagocytic lymphohistiocytosis (HLH). Bone marrow biopsy revealed infiltration with histoplasmosis and evidence of hemophagocytosis. No underlying cause of immunodeficiency including HIV was identified. He was treated initially with IV amphotericin and transitioned to posaconazole. Clinical syndrome of HLH resolved with treatment of histiocytosis.