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Insights from clinical studies into the role of the MLL gene in infant and childhood leukemia.
Blood Cells Mol Dis. 2008 Mar-Apr; 40(2):192-9.BC

Abstract

Translocations involving the Mixed Lineage Leukemia (MLL) gene at 11q23 are found in both acute lymphoblastic leukemia (ALL) and acute myeloblastic leukemia (AML), but have different prognostic implications depending on the phenotype of the leukemia in de novo pediatric cases. The majority of MLL gene rearrangements are associated with infant ALL, and their presence predicts a poor prognosis which worsens with earlier age of presentation. Rearrangements of the MLL gene are found in most cases of infant AML and regardless of age confer an intermediate risk. The treatment of MLL-rearranged ALL in children involves increased intensification of chemotherapy, and infants with ALL are treated with an intensive regimen of ALL- and AML-like chemotherapy, with the proportion of MLL-rearranged cases being responsible for the poor outcome in this age group. The use of DNA microarray analysis to distinguish a particular gene signature for MLL-rearranged leukemias is shedding light on the molecular mechanisms and potential therapeutic targets of these leukemias. It may also prove to have a useful role in both diagnosis and prognosis. This review considers recent advances in our understanding of the role of MLL gene rearrangements in pediatric clinical practice.

Authors+Show Affiliations

Molecular Haematology and Cancer Biology Unit, Institute of Child Health, University College London, London, UK.No affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't
Review

Language

eng

PubMed ID

17905612

Citation

Chowdhury, Tanzina, and Hugh J M. Brady. "Insights From Clinical Studies Into the Role of the MLL Gene in Infant and Childhood Leukemia." Blood Cells, Molecules & Diseases, vol. 40, no. 2, 2008, pp. 192-9.
Chowdhury T, Brady HJ. Insights from clinical studies into the role of the MLL gene in infant and childhood leukemia. Blood Cells Mol Dis. 2008;40(2):192-9.
Chowdhury, T., & Brady, H. J. (2008). Insights from clinical studies into the role of the MLL gene in infant and childhood leukemia. Blood Cells, Molecules & Diseases, 40(2), 192-9.
Chowdhury T, Brady HJ. Insights From Clinical Studies Into the Role of the MLL Gene in Infant and Childhood Leukemia. Blood Cells Mol Dis. 2008 Mar-Apr;40(2):192-9. PubMed PMID: 17905612.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Insights from clinical studies into the role of the MLL gene in infant and childhood leukemia. AU - Chowdhury,Tanzina, AU - Brady,Hugh J M, Y1 - 2007/10/01/ PY - 2007/07/17/received PY - 2007/07/19/accepted PY - 2007/10/2/pubmed PY - 2008/5/7/medline PY - 2007/10/2/entrez SP - 192 EP - 9 JF - Blood cells, molecules & diseases JO - Blood Cells Mol Dis VL - 40 IS - 2 N2 - Translocations involving the Mixed Lineage Leukemia (MLL) gene at 11q23 are found in both acute lymphoblastic leukemia (ALL) and acute myeloblastic leukemia (AML), but have different prognostic implications depending on the phenotype of the leukemia in de novo pediatric cases. The majority of MLL gene rearrangements are associated with infant ALL, and their presence predicts a poor prognosis which worsens with earlier age of presentation. Rearrangements of the MLL gene are found in most cases of infant AML and regardless of age confer an intermediate risk. The treatment of MLL-rearranged ALL in children involves increased intensification of chemotherapy, and infants with ALL are treated with an intensive regimen of ALL- and AML-like chemotherapy, with the proportion of MLL-rearranged cases being responsible for the poor outcome in this age group. The use of DNA microarray analysis to distinguish a particular gene signature for MLL-rearranged leukemias is shedding light on the molecular mechanisms and potential therapeutic targets of these leukemias. It may also prove to have a useful role in both diagnosis and prognosis. This review considers recent advances in our understanding of the role of MLL gene rearrangements in pediatric clinical practice. SN - 1079-9796 UR - https://www.unboundmedicine.com/medline/citation/17905612/Insights_from_clinical_studies_into_the_role_of_the_MLL_gene_in_infant_and_childhood_leukemia_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S1079-9796(07)00166-0 DB - PRIME DP - Unbound Medicine ER -