title: Intensified treatment of acute childhood lymphoblastic leukaemia has improved prognosis, especially in non-high-risk patients: the Nordic experience of 2648 patients diagnosed between 1981 and 1996. Nordic Society of Paediatric Haematology and Oncology (NOPHO)
abstract: In a multinational, population-based study from the five Nordic countries (Denmark, Finland, Iceland, Norway and Sweden), 2648 children below 15 y of age were diagnosed with acute lymphoblastic leukaemia (ALL) in the years 1981-1996. The annual incidence was 3.9/100000 children and was stable throughout the study period. The development from regional or national protocols to common Nordic treatment protocols for all risk groups was completed in 1992 through a successive intensification of therapy, based on multidrug chemotherapy including pulses of methotrexate in high doses and avoidance of cranial irradiation in most children. For children with non-B-cell ALL (n=2602), the event-free survival (p-EFS) increased from 0.53+/-0.02 (diagnosed 7/81-6/86) to 0.67+/-0.02 (7/86-12/91) to 0.78+/-0.02 (1/92-12/96). The corresponding p-EFS values at 5 y were 0.57, 0.70 and 0.78, respectively. The main improvements were seen in the group of children with non-high risk leukaemia, with 5-y p-EFS values increasing from 0.60 to 0.76 and 0.85 for the three periods. In high-risk patients, progress has been moderate, especially in children with high white blood cell values at diagnosis. During the last 5-y period, only 10% of the patients received cranial irradiation in first remission while 90% of the patients received high doses of cytostatic infusions (methotrexate isolated or combined with cytarabinoside) and multiple intrathecal injections of methotrexate as CNS-adjusted treatment without any indication of an increased CNS relapse rate.
authors.affiliation: Childhood Cancer Research Unit, Karolinska Hospital, Stockholm, Sweden.
authors.forename: G
authors.initials: G
authors.lastname: Gustafsson
authors.forename: A
authors.initials: A
authors.lastname: Kreuger
authors.forename: N
authors.initials: N
authors.lastname: Clausen
authors.forename: S
authors.initials: S
authors.lastname: Garwicz
authors.forename: J
authors.initials: J
authors.lastname: Kristinsson
authors.forename: S O
authors.initials: SO
authors.lastname: Lie
authors.forename: P J
authors.initials: PJ
authors.lastname: Moe
authors.forename: M
authors.initials: M
authors.lastname: Perkkiö
authors.forename: M
authors.initials: M
authors.lastname: Yssing
authors.forename: U M
authors.initials: UM
authors.lastname: Saarinen-Pihkala
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D002985:Clinical Protocols; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000188:drug therapy*; D011336:Probability; D011379:Prognosis; D012189:Retrospective Studies; D012537:Scandinavian and Nordic Countries; D016019:Survival Analysis; D016896:Treatment Outcome