Familial erythrophagocytic lymphohistiocytosis (FEL) is a rare disorder of the monocyte-macrophage system, for which an autosomal recessive mode of inheritance has been postulated. It is characterized by a dismal prognosis and is peculiar of early infancy. Three new cases of infants affected by FEL are reported. All three patients were diagnosed about three months after the onset of symptoms, and all three died shortly after diagnosis. The need for early diagnosis and prompt, intensive cytotoxic chemotherapy is emphasized.
Familial erythrophagocytic lymphohistiocytosis: adverse prognostic significance of delayed diagnosis / Fiorillo, Amedeo; Catera, P; Guarino, Alfredo; Grimaldi, M; Menna, G; Migliorati, Roberta; Vetrani, Antonio; Coletta, S; Monaco, G; De Chiara, C.. - In: HAEMATOLOGICA. - ISSN 0390-6078. - STAMPA. - 78:4(1993), pp. 242-244.
Familial erythrophagocytic lymphohistiocytosis: adverse prognostic significance of delayed diagnosis.
FIORILLO, AMEDEO;GUARINO, ALFREDO;MIGLIORATI, ROBERTA;VETRANI, ANTONIO;
1993
Abstract
Familial erythrophagocytic lymphohistiocytosis (FEL) is a rare disorder of the monocyte-macrophage system, for which an autosomal recessive mode of inheritance has been postulated. It is characterized by a dismal prognosis and is peculiar of early infancy. Three new cases of infants affected by FEL are reported. All three patients were diagnosed about three months after the onset of symptoms, and all three died shortly after diagnosis. The need for early diagnosis and prompt, intensive cytotoxic chemotherapy is emphasized.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
