Familial erythrophagocytic lymphohistiocytosis

A patient is described in whom the clinical symptoms and the laboratory data suggested familial erythrophagocytic lymphohistiocytosis (FEL). This diagnosis was confirmed by the findings on liver biopsy. The literature on FEL, a fatal disease with probable autosomal recessive inheritance, is reviewed. Some recently described aspects of FEL, such as hyperlipidemia and disturbances of the immune system, are stressed: the latter may play a role in the pathogenesis of the disease.