CBL mutation in childhood therapy-related leukemia.

摘要

Therapy-related leukemia and myelodysplastic syndrome (t-Leuk/MDS) are mainly caused by topoisomerase II inhibitors that cause acute myeloid leukemia (AML) with an 11 q23 translocation or by alkylating agents that induce MDS/AML with an AML 1 mutation and monosomy 7.1'2 Two types of t-Leuk/MDS can be distinguished, one of which has a long latency (>=5-7 years) and is seen following alkylating agents, frequently with an preleukemic phase. The other has a short latency period (1-3 years), no preleukemic phase, and is strongly associated with the administration of topoisomerase II inhibitors and chromosomal abnormalities involving 11q23 translocation/MLL. rearrangement (MLL-R). Repair of etoposide (VP-16)-stabilized DNA topoisomerase II covalent complexes may initiate MLL-R observed in patients.