title: [Malignant histiocytosis in children. Clinical and therapeutic aspects. Apropos of a series of 32 cases].
abstract: Thirty-two children with malignant histiocytosis were managed at the Department of Pediatrics of the Gustave-Roussy Institute from January 1975 through December 1987. Clinical presentation included enlarged lymph nodes in all cases, a decline in general health with fever in 74%, skin manifestations in 54%, and visceral involvement in 55%. Twenty-seven patients were treated with the COPAD protocol combining vincristine, cyclophosphamide, adriamycin and prednisone. Twenty-two patients achieved a complete remission, and one a partial remission; there was no remission in four patients. Eight patients had a recurrence and were given a combination of CCNU, vinblastine and bleomycin; seven achieved a second complete remission. Overall survival rate was 72.2% with a median follow up of five years. Survival rate free of disease was 54.5%. Poor prognostic factors included presence of constitutional symptoms upon diagnosis and age under ten years.
authors.forename: L
authors.initials: L
authors.lastname: Brugières
authors.forename: D
authors.initials: D
authors.lastname: Valteau
authors.forename: C
authors.initials: C
authors.lastname: Patte
authors.forename: C
authors.initials: C
authors.lastname: Kalifa
authors.forename: O
authors.initials: O
authors.lastname: Hartmann
authors.forename: J
authors.initials: J
authors.lastname: Lemerle
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D005260:Female; D054747:Histiocytic Sarcoma / Q000188:drug therapy* / Q000473:pathology; D006801:Humans; D007223:Infant; D008297:Male; D012189:Retrospective Studies
vernacular_title: L'histiocytose maligne de l'enfant. Aspects cliniques et thérapeutiques. A propos d'une série de trente-deux cas.