Dic(9;20)(p13;q11) in childhood acute lymphoblastic leukaemia is related to low cellular resistance to asparaginase, cytarabine and corticosteroids.

Abstract

Dic(9;20)(p13;q11) was first described as a nonrandom chromosome abnormality in B-cell precursor acute lymphoblastic leukaemia (BCP ALL) in the mid 1990s,1, 2 and 71 dic(9;20)-positive cases have since then been reported.3, 4, 5 Approximately 90% of these cases were children or adolescents, with dic(9;20) occurring in about 2% of childhood BCP ALL.6 The recent review by Forestier et al.5 describes that dic(9;20)-leukaemias are of B-cell precursor immunophenotype, never have a high hyperdiploid modal number, show a female predominance, and have a significant age incidence peak at 3 years. Most patients are allocated to non-standard risk treatment arms due to high WBC (median 24 109/l) and a relatively high frequency of CNS disease or other extra-medullary leukaemia (EML) at diagnosis. The prognostic implications of dic(9;20) are to a large extent unknown. A relatively large proportion of the relapses reported in the literature have been extra-medullary, and post-relapse treatment including block therapy has been successful in several patients, as illustrated by a p-EFS of 0.62 and a predicted overall survival of 0.82 at 5 years for the 24 Nordic cases.5