title: Outcome of very late relapse in children with acute lymphoblastic leukemia.
abstract: OBJECTIVE
  Few data are available on the long-term outcome of children who present with a very late relapse of acute lymphoblastic leukemia, so treatment of these patients remains controversial. The present study was aimed at investigating clinical features and treatment outcome of children with very late relapse, diagnosed and treated in Italy in the last 20 years.
  
  
  METHODS
  All children diagnosed in Italian centers with a first relapse of acute lymphoblastic leukemia occurring >or= 60 months after attainment of first complete remission were included in this study. These relapses were diagnosed between 1982 and 1997.
  
  
  RESULTS
  Ninety-three patients (58 males, 62.4%) had a first very late relapse occurring at a median time of 6.1 years (range 5.8 - 13.7 years) after the initial diagnosis. At a median follow-up time of 9.1 years after relapse, the overall 5-year survival (SE) and event-free-survival (SE) were 55.6% (5.2) and 39.5% (5.1), respectively. In multivariate analysis the site of relapse was the only significant predictor of duration of the second complete remission. Patients with isolated bone marrow relapse fared worse than those with combined or isolated extramedullary relapse [5-year event-free survival (SE) 24.5% (5.9), 51.3% (11.1) and 68.4% (10.7), respectively; (p=0.004)]. All 7 patients who underwent an allogeneic bone marrow transplantation from a matched related donor are alive in second complete remission.
  
  
  CONCLUSIONS
  In this evaluation patients with a very late relapse isolated to the bone marrow had a poor outcome while re-treatment of extramedullary or combined relapse was associated with better cure rate. Our data suggest that patients with very late isolated bone marrow relapse should be treated intensively; bone marrow transplantation from a matched related donor may be indicated.
authors.affiliation: Pediatric Department of Monza, Italy. carmelo.rizzari@pediatriamonza.it
authors.forename: Carmelo
authors.initials: C
authors.lastname: Rizzari
authors.forename: Maria Grazia
authors.initials: MG
authors.lastname: Valsecchi
authors.forename: Maurizio
authors.initials: M
authors.lastname: Aricò
authors.forename: Roberto
authors.initials: R
authors.lastname: Miniero
authors.forename: Chiara
authors.initials: C
authors.lastname: Messina
authors.forename: Giulio
authors.initials: G
authors.lastname: De Rossi
authors.forename: Anna Maria
authors.initials: AM
authors.lastname: Testi
authors.forename: Rossella
authors.initials: R
authors.lastname: Mura
authors.forename: Stefania
authors.initials: S
authors.lastname: Galimberti
authors.forename: Andrea
authors.initials: A
authors.lastname: Biondi
authors.forename: Franco
authors.initials: F
authors.lastname: Locatelli
authors.forename: Valentino
authors.initials: V
authors.lastname: Conter
mesh_terms: D000293:Adolescent; D001853:Bone Marrow / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007558:Italy / Q000453:epidemiology; D008297:Male; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000175:diagnosis* / Q000401:mortality; D011379:Prognosis; D012008:Recurrence; D012074:Remission Induction; D012189:Retrospective Studies; D016896:Treatment Outcome