Les leucémies aiguës lymphoblastiques congénitales : expérience française entre 1990 et 2010

Purpose. - Our aim was to describe acute lymphoblastic leukemia diagnosed in the first month of life, in a comprehensive way and to analyze the modalities of care and the ethical approach.Methods. - A retrospective national register- based study of congenital acute lymphoblastic leukemia cases from 1990 to 2010, was performed. Clinical and biological characteristics, treatment modalities, outcome and prognosis factors were studied.Results. - Twenty-two children were included, with a median age at diagnosis of 5.5 days. A mixed lineage leukemia gene rearrangement or 11q23 abnormalities was present in 17/22 cases. Fourteen patients were treated in intention to cure whereas seven received supportive cares only. The treatment decision was delayed for three. The prognosis was very poor, with a twoyear overall survival rate of 4.5%; only one child survived with heavy neurological sequelae. Additional unfavorable prognostic factors (P < 0.05) were age (< 8 days to diagnosis), poor steroid response at day seven of treatment and induction failure.Conclusion. - In this study, congenital acute lymphoblastic leukemia appeared to occur earlier and to be more severe than in clinical trials where infants were included selectively. This extremely poor prognosis requires including ethical considerations in the therapeutic strategy, aiming to preserve the comfort of the child and to support attachment behavior or grieving process for the parents and family. (C) 2014 Elsevier Masson SAS. All rights reserved.