A. Fiorillo et al., FAMILIAL ERYTHROPHAGOCYTIC LYMPHOHISTIOCYTOSIS - ADVERSE PROGNOSTIC-SIGNIFICANCE OF DELAYED DIAGNOSIS, Haematologica, 78(4), 1993, pp. 242-244
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.