ACUTE MYELOGENOUS LEUKEMIA IN DOWNS-SYNDROME - REPORT OF A SINGLE PEDIATRIC INSTITUTION USING A BFM TREATMENT STRATEGY

Between July 1990 and December 1995, 111 new consecutive pediatric pat ients with acute myelogenous leukemia (AML) have been treated in our i nstitution. Eleven of them (9.9%) had Down's syndrome (DS), 6 boys and 5 girls. The median age was 22.5 (range 10-40) months. FAB subtypes w ere the following: M7: 6, M4: 3, and M0: 2. Five of them had previousl y had myelodysplasia and in 3, all FAB M7, myelofibrosis was detected. This population was treated with two consecutive protocols. Nine pati ents were included in the AML-HPG-90 protocol and 2 patients in the AM L-HPG-95 study, respectively. However, all DS patients in this series received the same treatment. Eight patients achieved complete remissio n: two patients received two cycles of intensification with high dose (HD) ara-C, and 1 patient, only one cycle; the other 5 were prevented from receiving such therapy because of unacceptable toxicity or death. At 45 months, event-free survival and overall survival estimates were 0.30, S.E. 0.16. Mortality was remarkably high. All deaths (7) were a ssociated with sepsis (5) or pulmonary infection (2). Three deaths occ urred before achieving complete remission, 3 patients died during the consolidation phase and 1 died whilst off treatment. No one presented leukemic relapse. We conclude that this AML-BFM treatment strategy is highly toxic to children with DS and AML in our setting. Efforts will be made to improve clinical support and to administer less intensive t herapy to this particular pediatric AML subgroup, which, in fact, has a better prognosis than the same non-trisomic population. (C) 1998 Els evier Science Ltd. All rights reserved.