Details

Autor(en) / Beteiligte

• DE BOTTON, S
• COITEUX, V
• ROBERT, A
• J. SAN MIGUEL
• CONDE, E
• SOTTO, J. J
• BORDESSOULE, D
• FEGUEUX, N
• FEY, M
• PARRY, A
• CHOMIENNE, C
• DEGOS, L
• CHEVRET, S
• FENAUX, P
• RAYON, C
• VILMER, E
• SANZ, M
• DE LA SERNA, J
• PHILIPPE, N
• BORUCHEL, A
• LEVERGER, G

Titel

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

Ist Teil von

• Journal of clinical oncology, 2004-04, Vol.22 (8), p.1404-1412

Ort / Verlag

Baltimore, MD: American Society of Clinical Oncology

Erscheinungsjahr

2004

Quelle

MEDLINE

Beschreibungen/Notizen

• To determine the results of treatment combining all-trans-retinoic acid (ATRA) and chemotherapy (CT) in childhood acute promyelocytic leukemia (APL). 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. 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). 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.