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Prepublished online as a Blood First Edition Paper on May 15, 2003; DOI 10.1182/blood-2002-07-1960.

Submitted July 2, 2002
Accepted May 3, 2003
Peripheral T-cell lymphomas unspecified presenting in the skin: analysis of prognostic factors in a group of 82 patients
Marcel W Bekkenk, Maarten H Vermeer, Patty M Jansen, Arienne M W van Marion, Marijke R Canninga-van Dijk, Philip M Kluin, Marie-Louise Geerts, Chris J L M Meijer, and Rein Willemze*
Department of Dermatology, Leiden University Medical Center, Leiden, The Netherlands
Department of Pathology, Leiden University Medical Center, Leiden, The Netherlands
Department of Pathology, University Medical Center Utrecht, Utrecht, The Netherlands
Department of Pathology, University Hospital Gronongen, Groningen, The Netherlands
Department of Pathology, Vrije Universiteit Medical Center, Amsterdam, The Netherlands
Department of Dermatology, University Hospital Gent, Gent, Belgium
* Corresponding author; email: willemze.dermatology{at}lumc.nl.
In the present study the clinicopathologic and immunophenotypical features of 82 patients with a CD30-negative peripheral T-cell lymphoma, unspecified, presenting in the skin were evaluated. The purpose of this study was to find out if subdivision of these lymphomas on the basis of presentation with only skin lesions, cell size or phenotype is clinically relevant. The study group included 46 primary cutaneous CD30-negative large cell lymphomas and 17 small/medium-sized T-cell lymphomas as well as 17 peripheral T-cell lymphoma with both skin and extracutaneous disease at the time of diagnosis. Patients with a primary cutaneous small/medium-sized T-cell lymphoma had a significantly better prognosis (5-year-overall survival, 45%) than patients with a primary cutaneous CD30-negative large T-cell lymphomas (12%) and patients presenting with concurrent extracutaneous disease (12%). The favorable prognosis in this primary cutaneous small/medium-sized group was particularly found in patients presenting with localized skin lesions expressing a CD3+, CD4+, CD8- phenotype. In the primary cutaneous large T-cell lymphomas and in the concurrent group neither extent of skin lesions nor phenotype had any effect on survival. Our results indicate that peripheral T-cell lymphomas, unspecified, presenting in the skin have an unfavorable prognosis, irrespective of the presence or absence of extracutaneous disease at the time of diagnosis, cell size and expression of a CD4+ or CD8+ phenotype. The only exception is a group of primary cutaneous small/medium-sized pleomorphic CTCL with a CD3+, CD4+, CD8- phenotype and presenting with localized skin lesions.

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