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. 2011 Sep;25(9):1494-501.
doi: 10.1038/leu.2011.101. Epub 2011 May 31.

Tumor suppressor TNFAIP3 (A20) is frequently deleted in Sézary syndrome

F C M Braun  1 , P Grabarczyk, M Möbs, F K Braun, J Eberle, M Beyer, W Sterry, F Busse, J Schröder, M Delin, G K Przybylski, C A Schmidt
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Tumor suppressor TNFAIP3 (A20) is frequently deleted in Sézary syndrome

F C M Braun et al. Leukemia. 2011 Sep.

Abstract

Despite recent therapeutic improvements, the prognosis for patients suffering from Sézary syndrome (SS), a disseminated form of cutaneous T-cell lymphomas, is still poor. We identified bi- and monoallelic deletions of the tumor necrosis factor-α-induced protein 3 gene (TNFAIP3; A20) in a high proportion of SS patients as well as biallelic A20 deletion in the SS-derived cell line SeAx. Furthermore, we demonstrate that inhibition of A20 activates the NF-κB pathway thereby increasing the proliferation of normal T lymphocytes. On the other hand, the reconstitution of A20 expression slowed down the cell cycle in SeAx cells. Recently A20 inactivation has been reported in various B-cell lymphomas. In this study, we show that A20 is also a putative tumor suppressor in the T-cell malignancy-SS.

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