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GIORNALE ITALIANO DI DERMATOLOGIA E VENEREOLOGIA

A Journal on Dermatology and Sexually Transmitted Diseases


Official Journal of the Italian Society of Dermatology and Sexually Transmitted Diseases
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Giornale Italiano di Dermatologia e Venereologia 2005 June;140(3):249-61

language: English, Italian

Natural killer and T-cell cytotoxic cutaneous lymphomas

Berti E. 1, Gambini D. 2, Lucioni M. 3, Paulli M. 3

1 Department of Dermatology IRCCS, University of Milano-Bicocca, Milan, Italy
2 Department of Dermatology IRCCS, University of Milan, Milan, Italy
3 Department of Pathological Anatomy IRCCS San Matteo Hospital University of Pavia, Pavia, Italy


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In the group of primary cutaneous T-cell lymphomas, T-cytotoxic and natural killer cell lymphomas have been recently recognized as distinct and rare clinicopathologic entities. Typically, cytotoxic granules associated proteins TIA-1, granulolysine and perforin, and cytotoxic-natural killer (NK) cell markers CD8+, CD56+, CD94+, CD161+, NKp46+ are expressed by tumoral cells. Five T-cytotoxic and NK cutaneous lymphomas subtypes are actually recognized: subcutaneous panniculitis-like T-cell lymphoma, extranodal nasal and nasal-type NK-T cell lymphoma, blastic NK cell lymphoma, aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma, cutaneous gamma-delta peripheral T-cell lymphoma. In this review, the most important clinical, histological, immunohistochemical and molecular data are analyzed together with differential diagnostic aspects between these entities. T-cytotoxic and NK cutaneous lymphomas pursue a very aggressive course, with poor outcome and high mortality rate, with few exceptions.

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