[Familial erythrophagocytic lymphohistiocytosis (author's transl)].

A four-year old boy with familial erythrophagocytic lymphohistiocytosis is reported. The clinical picture was that of prolonged fever, hepato and splenomegaly, adenopathies and, in the terminal state, bleeding tendency and sligth jaundice. The laboratory data showed anemia, leucopenia and abnormal coagulation studies compatibles with disseminated intravascular clotting. The differential diagnosis with malignant histiocytosis is attempted from a histological point of view.