Haematologica
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Published online 19 May 2008
Haematologica, Vol 93, Issue 7, 1081-1085 doi:10.3324/haematol.12595
Copyright © 2008 by Ferrata Storti Foundation
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Lymphoproliferative Disorders

Transcriptional activation of the cardiac homeobox gene CSX1/NKX2-5 in a B-cell chronic lymphoproliferative disorder

Xinying Su1,5, Véronique Della-Valle1,5, Eric Delabesse3, Zahia Azgui2, Roland Berger1, Hélène Merle-Béral2,4, Olivier A Bernard1,5, Florence Nguyen-Khac1,2,4

1 INSERM, E0210, Hôpital Necker, Paris
2 Service d’Hematologie Biologique, Groupe Hospitalier Pitie-Salpetriere, Paris
3 Laboratoire d’Hématologie, CHU Purpan, Toulouse
4 Université Pierre et Marie Curie, Paris VI
5 Université Rene Descartes, Paris V, France

Correspondence: Florence Nguyen-Khac, Service d’Hematologie Biologique Groupe Hospitalier Pitié-Salpetriére, Pavillon Laveran, 47-83 Bd de l’Hopital, 75013 Paris, France. E-mail:florence.nguyen{at}psl.aphp.fr.

Homeobox containing transcription factors are frequently deregulated in human hematologic malignant diseases either indirectly through an abnormality of an upstream factor, or directly through rearrangement of the gene itself. Study of T-cell acute lymphoblastic leukemia identified the related non-clustered homeobox transcription factors, TLX1 and TLX3, as frequently ectopically expressed as a result of chromosomal translocations. We report the deregulation of a non-clustered homeobox gene in a new type of t(5;14)(q35;q11) translocation in a mature peripheral B-cell leukemia. This translocation results in the ectopic expression of the CSX1/NKX2-5 gene on chromosome 5q35 due to its juxtaposition to the TCR {delta} gene on chromosome 14q11. Expression of the CSX1/NKX2-5 protein conferred enhanced replating potential to transduced murine bone marrow cells. Our study establishes that deregulation of homeobox encoding genes is not restricted to acute leukemic proliferations, but is also observed in chronic malignant diseases.

Key words: chromosomal translocation, homeobox, transcriptional activation, chronic lymphocytic leukemia.






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