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Advances in Hematology
Volume 2011, Article ID 621627, 8 pages
http://dx.doi.org/10.1155/2011/621627
Review Article

Pathogenetic, Clinical, and Prognostic Features of Adult t(4;11)(q21;q23)/MLL-AF4 Positive B-Cell Acute Lymphoblastic Leukemia

Unit of Hematology, Stem Cell Transplantation, Transfusion Medicine and Cellular Therapy, Campus Bio-Medico University Hospital, Via Àlvaro del Portillo 200, 00128 Rome, Italy

Received 15 June 2011; Revised 23 August 2011; Accepted 6 September 2011

Academic Editor: Peter J. Quesenberry

Copyright © 2011 F. Marchesi et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Translocation t(4;11)(q21;q23) leading to formation of MLL-AF4 fusion gene is found in about 10% of newly diagnosed B-cell acute lymphoblastic leukemia (ALL) in adult patients. Patients expressing this chromosomal aberration present typical biological, immunophenotypic, and clinical features. This form of leukemia is universally recognized as high-risk leukemia and treatment intensification with allogeneic hematopoietic stem cell transplantation (HSCT) in first complete remission (CR) could be a valid option to improve prognosis, but data obtained from the literature are controversial. In this review, we briefly describe pathogenetic, clinical, and prognostic characteristics of adult t(4;11)(q21;q23)/MLL-AF4 positive ALL and provide a review of the clinical outcome reported by the most important cooperative groups worldwide.