B-cell precursor (BCP) acute lymphoblastic leukemia (BCP-ALL) in infants (ie, children age <1 year) is a rare disease traditionally subdivided into MLL-rearranged (alias KMT2A; MLL-R) and MLL germ line (MLL-G) subtypes. MLL gene rearrangements occur in ∼75% of infants with BCP-ALL1-3 and are typically associated with a mixed-lineage phenotype.4 MLL rearrangements originate prenatally in utero5 and play a role in transcription factor dysregulation.6 MLL-R BCP-ALL in infants is associated with dismal outcomes.1-3 Infants with MLL-G ALL treated with intensive therapies in the Interfant-06 protocol may have a relatively favorable prognosis, with a 6-year event-free survival (EFS) rate of 73.9%,2 slightly inferior to that observed in older children with BCP-ALL.1,2 Of interest, a recent study from the Japanese Pediatric Leukemia/Lymphoma Study Group MLL-10 trial reported more favorable outcomes in infants
Fazio, G., Bardini, M., De Lorenzo, P., Grioni, A., Quadri, M., Pedace, L., et al. (2021). Recurrent genetic fusions redefine MLL germ line acute lymphoblastic leukemia in infants. BLOOD, 137(14), 1980-1984 [10.1182/blood.2020009032].
Recurrent genetic fusions redefine MLL germ line acute lymphoblastic leukemia in infants
Fazio G.;Bardini M.;Grioni A.;Quadri M.;Palmi C.;Conter V.;Rizzari C.;Valsecchi M. G.;Biondi A.;Cazzaniga G.
2021
Abstract
B-cell precursor (BCP) acute lymphoblastic leukemia (BCP-ALL) in infants (ie, children age <1 year) is a rare disease traditionally subdivided into MLL-rearranged (alias KMT2A; MLL-R) and MLL germ line (MLL-G) subtypes. MLL gene rearrangements occur in ∼75% of infants with BCP-ALL1-3 and are typically associated with a mixed-lineage phenotype.4 MLL rearrangements originate prenatally in utero5 and play a role in transcription factor dysregulation.6 MLL-R BCP-ALL in infants is associated with dismal outcomes.1-3 Infants with MLL-G ALL treated with intensive therapies in the Interfant-06 protocol may have a relatively favorable prognosis, with a 6-year event-free survival (EFS) rate of 73.9%,2 slightly inferior to that observed in older children with BCP-ALL.1,2 Of interest, a recent study from the Japanese Pediatric Leukemia/Lymphoma Study Group MLL-10 trial reported more favorable outcomes in infants
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