title: Clinico-biological features of 5202 patients with acute lymphoblastic leukemia enrolled in the Italian AIEOP and GIMEMA protocols and stratified in age cohorts.
abstract: The outcome of children and adults with acute lymphoblastic leukemia is markedly different. Since there is limited information on the distribution of clinico-biological variables in different age cohorts, we analyzed 5202 patients with acute lymphoblastic leukemia enrolled in the Italian multicenter AIEOP and GIMEMA protocols and stratified them in nine age cohorts. The highest prevalence of acute lymphoblastic leukemia was observed in children, although a second peak was recorded from the 4(th) decade onwards. Interestingly, the lowest incidence was found in females between 14-40 years. Immunophenotypic characterization showed a B-lineage in 85.8% of patients: a pro-B stage, associated with MLL/AF4 positivity, was more frequent in patients between 10-50 years. T-lineage leukemia (14.2%) was rare among small children and increased in patients aged 10-40 years. The prevalence of the BCR/ABL1 rearrangement increased progressively with age starting from the cohort of patients 10-14 years old and was present in 52.7% of cases in the 6th decade. Similarly, the MLL/AF4 rearrangement constantly increased up to the 5(th) decade, while the ETV6/RUNX1 rearrangement disappeared from the age of 30 onwards. This study shows that acute lymphoblastic leukemia in adolescents and young adults is characterized by a male prevalence, higher percentage of T-lineage cases, an increase of poor prognostic molecular markers with aging compared to cases in children, and conclusively quantified the progressive increase of BCR/ABL(+) cases with age, which are potentially manageable by targeted therapies.
authors.affiliation: rfoa@bce.uniroma1.it or chiaretti@bce.uniroma1.it.
authors.forename: Sabina
authors.initials: S
authors.lastname: Chiaretti
authors.forename: Antonella
authors.initials: A
authors.lastname: Vitale
authors.forename: Gianni
authors.initials: G
authors.lastname: Cazzaniga
authors.forename: Sonia Maria
authors.initials: SM
authors.lastname: Orlando
authors.forename: Daniela
authors.initials: D
authors.lastname: Silvestri
authors.forename: Paola
authors.initials: P
authors.lastname: Fazi
authors.forename: Maria Grazia
authors.initials: MG
authors.lastname: Valsecchi
authors.forename: Loredana
authors.initials: L
authors.lastname: Elia
authors.forename: Anna Maria
authors.initials: AM
authors.lastname: Testi
authors.forename: Francesca
authors.initials: F
authors.lastname: Mancini
authors.forename: Valentino
authors.initials: V
authors.lastname: Conter
authors.forename: Geertruy
authors.initials: G
authors.lastname: te Kronnie
authors.forename: Felicetto
authors.initials: F
authors.lastname: Ferrara
authors.forename: Francesco
authors.initials: F
authors.lastname: Di Raimondo
authors.forename: Alessandra
authors.initials: A
authors.lastname: Tedeschi
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Fioritoni
authors.forename: Francesco
authors.initials: F
authors.lastname: Fabbiano
authors.forename: Giovanna
authors.initials: G
authors.lastname: Meloni
authors.forename: Giorgina
authors.initials: G
authors.lastname: Specchia
authors.forename: Giovanni
authors.initials: G
authors.lastname: Pizzolo
authors.forename: Franco
authors.initials: F
authors.lastname: Mandelli
authors.forename: Anna
authors.initials: A
authors.lastname: Guarini
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Basso
authors.forename: Andrea
authors.initials: A
authors.lastname: Biondi
authors.forename: Robin
authors.initials: R
authors.lastname: Foà
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D006801:Humans; D007223:Infant; D007558:Italy / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000175:diagnosis* / Q000453:epidemiology* / Q000235:genetics; D012189:Retrospective Studies; D055815:Young Adult