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 Blood, 15 November 2006, Vol. 108, No. 10, pp. 3560-3563.
Prepublished online as a Blood First Edition Paper on July 27, 2006; DOI 10.1182/blood-2006-03-010835.

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NEOPLASIA
Brief report

Overexpression of CEBPA resulting from the translocation t(14;19)(q32;q13) of human precursor B acute lymphoblastic leukemia

Elise Chapiro, Lisa Russell, Isabelle Radford-Weiss, Christian Bastard, Michel Lessard, Stephanie Struski, Helene Cave, Sandra Fert-Ferrer, Carole Barin, Odile Maarek, Veronique Della-Valle, Jonathan C. Strefford, Roland Berger, Christine J. Harrison, Olivier A. Bernard, Florence Nguyen-Khac, the Groupe Francophone de Cytogénétique Hématologique

From the Assistance Publique-Hôpitaux de Paris (AP-HP) Service d'Hématologie Biologique, Hôpital Pitié-Salpêtrière, Paris, France; Institut National de la Santé etde la Recherche Médicale (INSERM), E0210, Paris, France; University Paris VI, Paris, France; Leukaemia Research Cytogenetics Group, Cancer Sciences Division, University of Southampton, United Kingdom; University René Descartes, Paris, France; AP-HP, Laboratoire de Cytogénétique, Hôpital Necker-Enfants Malades, Paris, France; Laboratoire de Génétique oncologique, Rouen, France; Laboratoire d'Hématologie, Hôpital Hautepierre, Strasbourg, France; AP-HP, Laboratoire de Biochimie Génétique, Hôpital Robert Debré, Paris, France; Laboratoire de Génétique, Chambéry, France; Laboratoire de Génétique, Tours, France; and AP-HP, Laboratoire d'Hématologie, Hôpital Saint-Louis, Paris, France.

Subtle variation in the expression or function of a small group of transcription factors can drive leukemogenesis. The CEBPA protein is known to regulate the balance between cell proliferation and differentiation during early hematopoietic development and myeloid differentiation. In human myeloid leukemia, CEBPA is frequently inactivated by mutation and indirect and posttranslational mechanisms, in keeping with tumor suppressor properties. We report that CEBPA is activated by juxtaposition to the immunoglobulin gene enhancer upon its rearrangement with the immunoglobulin heavy-chain locus in precursor B-cell acute lymphoblastic leukemia harboring t(14;19)(q32;q13). Overexpression of apparently normal CEBPA RNA or protein was observed in 6 patients. These data indicate that CEBPA may exhibit oncogenic as well as tumor suppressor properties in human leukemogenesis.


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