Familial erythrophagocytic lymphohistiocytosis: adverse prognostic significance of delayed diagnosis.
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.
Fiorillo
A
A
Department of Pediatrics, Faculty of Medicine, University of Naples Federico II, Italy.
Catera
P
P
Guarino
A
A
Grimaldi
M
M
Menna
G
G
Migliorati
R
R
Vetrani
A
A
Coletta
S
S
Monaco
G
G
De Chiara
C
C
D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D017809:Fatal Outcome; D005260:Female; D015614:Histiocytosis / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics* / Q000401:mortality; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007223:Infant; D008297:Male; D010587:Phagocytosis; D011379:Prognosis; D013997:Time Factors
D007166:Immunosuppressive Agents