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Molecular and cytogenetic characterization of a new case of t(5;17)(q35;q21) variant acute promyelocytic leukemia

Leukemia volume 19, pages 470–472 (2005)Cite this article

TO THE EDITOR

Acute promyelocytic leukemia is characterized by reciprocal chromosomal translocation t(15;17)(q22;q21). This translocation results in the fusion of the retinoic acid receptor (RARα) gene to the promyelocytic leukemia gene (PML).1 In rare APL cases, variant chromosome translocations have been reported, which fuse RARα gene with partner genes other than PML, such as t(11;17)(q23;q21),2 t(5;17)(q35;q21),3 t(11;17)(q13;q21) and dup(17)(q21.3;q23).2 In these cases RARα is fused to the PLZF, NPM, NuMA and STAT5b genes, respectively. Patients with fusion genes involving NPM and NuMA appear to be sensitive to ATRA, like patients with PML-RARα rearrangement.1,4 In contrast, APL with a PLFZ-RARα rearrangement lack differentiation response to retinoids, and the patients treated with ATRA alone have a poor prognosis.1 The t(5;17) translocation is a very rare variant and leads to the NPM-RARα fusion protein and the reciprocal RARα-NPM protein.5 NPM (nucleophosmin) is a nucleolar phosphoprotein that plays a role in ribosomal RNA assembly; it also has chaperoning activities, as well as nuclease activity. Recently, it has been found to associate with centrosomes, and possibly plays a role in the regulation of centrosomal duplication.6 NPM-RARα protein has been shown to act as ligand-dependent transcriptional activator.7

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Acknowledgements

This work was supported by the University of Bologna (ex-60% RFO), by Fondazione del Monte di Bologna e Ravenna and by COFIN 2003.

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  1. Institute of Hematology and Medical Oncology ‘Lorenzo e Ariosto Seràgnoli’, University of Bologna, Bologna, Italy

    C Nicci, E Ottaviani, S Luatti, T Grafone, M Tonelli, M R Motta, M Malagola, G Marzocchi, G Martinelli, M Baccarani & N Testoni

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Nicci, C., Ottaviani, E., Luatti, S. et al. Molecular and cytogenetic characterization of a new case of t(5;17)(q35;q21) variant acute promyelocytic leukemia. Leukemia 19, 470–472 (2005). https://doi.org/10.1038/sj.leu.2403645

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