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Outcome of Childhood Acute Promyelocytic Leukemia With All- Trans -Retinoic Acid and Chemotherapy

2004; Lippincott Williams & Wilkins; Volume: 22; Issue: 8 Linguagem: Inglês

10.1200/jco.2004.09.008

1527-7755

Stéphane de Botton, Valérie Coiteux, Sylvie Chevret, Chelo Rayón, E Vilmer, Miguel Á. Sanz, Javier de la Serna, N Philippe, André Baruchel, Guy Leverger, A Robert, Jesús F. San Miguel, E Conde, J.J. Sotto, Dominique Bordessoule, Nathalie Fegueux, Martin F. Fey, Anne Parry, Christine Chomienne, L Degos, Pierre Fenaux,

Ocular Oncology and Treatments

Purpose To determine the results of treatment combining all-trans-retinoic acid (ATRA) and chemotherapy (CT) in childhood acute promyelocytic leukemia (APL). Patients and Methods Children (< 18 years) with newly diagnosed APL were included in the APL93 trial, treated by ATRA followed or combined with daunorubicin-cytarabine, and then randomly assigned between no maintenance, intermittent ATRA, continuous CT, or both. Results Of the 576 patients included in APL93 trial, 31 (5%) were children, including 22 girls (71%) and nine boys (29%). Thirty of the children (97%) obtained complete remission (CR). ATRA syndrome occurred in four children (13%), who all achieved CR, and headaches occurred in 12 children (39%), with signs of pseudotumor cerebri in five children (16%). Seven patients (23%) relapsed. None of the eight patients who received both ATRA and CT for maintenance relapsed. All relapsing patients achieved a second CR. Twenty-two patients remained in first CR after 43+ to 96+ months, six remained in second CR after 17+ to 66+ months, and three patients had died. The 5-year event-free survival (EFS), relapse, and overall survival rates were 71%, 27%, and 90%, respectively. No difference between adults and children included in the APL93 trial was seen for CR rate, 5-year relapse rate, EFS, and overall survival, but significantly better survival was seen in children after adjustment on WBC counts (P = .02) and incidence of microgranular M3 variant (P = .04). Conclusion ATRA combined with CT for induction and also probably for maintenance provides as favorable results in children with APL as in adults and currently constitutes the reference first-line treatment in both age groups.