title: ATRA and anthracycline-based chemotherapy in the treatment of childhood acute promyelocytic leukemia (APL): A 10-year experience in Tunisia.
abstract: Reports on childhood APL from developing countries are scarce. We treated 65 APL with two consecutive trials combining ATRA and chemotherapy. Twenty (30.7%) were aged less than 20 years including 11 girls and 9 boys, with a median age of 12 years. Fever at presentation (P=0.002) and variant APL (P=0.044) were more frequent in children, while there were no significant difference between children and adults for WBC count, Sanz's score distribution and additional cytogenetic abnormalities. The CR rate was 95% (19/20) in children and 80% (36/45) in adults (P=0.13). Differentiation syndrome (DS) was less often observed in children (1/20) than in adults (13/45) (P=0.031). Two children relapsed and died during salvage therapy, and 2 died in CR from infection and from cardiac failure attributed to anthracyclines, while other children remained alive in CR. With a median follow-up of 4 years, 4-year EFS was 75% in children and 71.1% in adults (P=0.57), while 4-year OS was 75% in children vs. 73.3% in adults (P=0.72). Our results suggest that, even in the absence of optimal socio-economic condition, ATRA combined with anthracycline-based chemotherapy gives adequate results in childhood APL, as in adults.
authors.affiliation: Hematology Department, Aziza Othmana University Hospital, Tunis, Tunisia. ramzi.jed@voila.fr
authors.forename: Ramzi
authors.initials: R
authors.lastname: Jeddi
authors.forename: Hèlà
authors.initials: H
authors.lastname: Ghédira
authors.forename: Yosr
authors.initials: Y
authors.lastname: Ben Abdennebi
authors.forename: Karima
authors.initials: K
authors.lastname: Kacem
authors.forename: Ramzi
authors.initials: R
authors.lastname: Ben Amor
authors.forename: Lamia
authors.initials: L
authors.lastname: Aissaoui
authors.forename: Walid
authors.initials: W
authors.lastname: Bouterâa
authors.forename: Raihane
authors.initials: R
authors.lastname: Ben Lakhal
authors.forename: Hèla
authors.initials: H
authors.lastname: Ben Abid
authors.forename: Samia
authors.initials: S
authors.lastname: Menif
authors.forename: Zaher
authors.initials: Z
authors.lastname: Belhadjali
authors.forename: Balkis
authors.initials: B
authors.lastname: Meddeb
mesh_terms: D000293:Adolescent; D000328:Adult; D018943:Anthracyclines / Q000008:administration & dosage*; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D053208:Kaplan-Meier Estimate; D015473:Leukemia, Promyelocytic, Acute / Q000188:drug therapy* / Q000401:mortality*; D008297:Male; D008875:Middle Aged; D016896:Treatment Outcome; D014212:Tretinoin / Q000008:administration & dosage*; D014416:Tunisia; D055815:Young Adult
chemical_list: D018943:Anthracyclines; D014212:Tretinoin