Title: PRDM1/BLIMP1 is commonly inactivated in anaplastic large T-cell lymphoma
Authors: Boi, Michela ×
Rinaldi, Andrea
Kwee, Ivo
Bonetti, Paola
Todaro, Maria
Tabbò, Fabrizio
Piva, Roberto
Rancoita, Paola M V
Matolcsy, András
Timar, Botond
Tousseyn, Thomas
Rodríguez-Pinilla, Socorro Maria
Piris, Miguel A
Beà, Sílvia
Campo, Elias
Bhagat, Govind
Swerdlow, Steven H
Rosenwald, Andreas
Ponzoni, Maurilio
Young, Ken H
Piccaluga, Pier Paolo
Dummer, Reinhard
Pileri, Stefano
Zucca, Emanuele
Inghirami, Giorgio
Bertoni, Francesco #
Issue Date: 10-Oct-2013
Publisher: W.B. Saunders
Series Title: Blood vol:122 issue:15 pages:2683-2693
Article number: 10.1182/blood-2013-04-497933
Abstract: Anaplastic large cell lymphoma (ALCL) is a mature T-cell lymphoma that can present as a systemic or primary cutaneous disease. Systemic ALCL represents 2% to 5% of adult lymphoma but up to 30% of all pediatric cases. Two subtypes of systemic ALCL are currently recognized on the basis of the presence of a translocation involving the anaplastic lymphoma kinase ALK gene. Despite considerable progress, several questions remain open regarding the pathogenesis of both ALCL subtypes. To investigate the molecular pathogenesis and to assess the relationship between the ALK(+) and ALK(-) ALCL subtypes, we performed a genome-wide DNA profiling using high-density, single nucleotide polymorphism arrays on a series of 64 cases and 7 cell lines. The commonest lesions were losses at 17p13 and at 6q21, encompassing the TP53 and PRDM1 genes, respectively. The latter gene, coding for BLIMP1, was inactivated by multiple mechanisms, more frequently, but not exclusively, in ALK(-)ALCL. In vitro and in vivo experiments showed that that PRDM1 is a tumor suppressor gene in ALCL models, likely acting as an antiapoptotic agent. Losses of TP53 and/or PRDM1 were present in 52% of ALK(-)ALCL, and in 29% of all ALCL cases with a clinical implication.
ISSN: 0006-4971
Publication status: published
KU Leuven publication type: IT
Appears in Collections:Translational Cell & Tissue Research
× corresponding author
# (joint) last author

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