FAMILIAL ERYTHROPHAGOCYTIC LYMPHOHISTIOCYTOSIS - ADVERSE PROGNOSTIC-SIGNIFICANCE OF DELAYED DIAGNOSIS

Familial erythrophagocytic lymphohistiocytosis (FEL) is a rare disorde r of the monocyte-macrophage system, for which an autosomal recessive mode of inheritance has been postulated. It is characterized by a dism al prognosis and is peculiar of early infancy. Three new cases of infa nts affected by FEL are reported. All three patients were diagnosed ab out three months after the onset of symptoms, and all three died short ly after diagnosis. The need for early diagnosis and prompt, intensive cytotoxic chemotherapy is emphasized.