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Prepublished online as a Blood First Edition Paper on May 15, 2003; DOI 10.1182/blood-2002-07-1960.
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Blood, 15 September 2003, Vol. 102, No. 6, pp. 2213-2219
NEOPLASIA
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,
Ariënne M. W. van Marion,
Marijke R. Canninga-van Dijk,
Philip M. Kluin,
Marie-Louise Geerts,
Chris J. L. M. Meijer, and
Rein Willemze
From the Department of Dermatology, Leiden University Medical Center, Leiden, the Netherlands; the Department of Pathology, Leiden University Medical Center, Leiden, the Netherlands; the Department of Pathology, University Medical Center Utrecht, Utrecht, the Netherlands; Department of Pathology, University of Groningen, Groningen, the Netherlands; Department of Pathology, Vrije Universiteit Medical Center, Amsterdam, Netherlands; and the Department of Dermatology, University Hospital Gent, Gent, Belgium.
In the present study the clinicopathologic and immunophenotypic features of 82 patients with a CD30– peripheral T-cell lymphoma, unspecified, presenting in the skin were evaluated. The purpose of this study was to find out whether subdivision of these lymphomas on the basis of cell size, phenotype, or presentation with only skin lesions is clinically relevant. The study group included 46 primary cutaneous CD30– large cell lymphomas and 17 small/medium-sized T-cell lymphomas as well as 17 peripheral T-cell lymphomas with both skin and extracutaneous disease at the time of diagnosis. Patients with primary cutaneous small- or medium-sized T-cell lymphomas had a significantly better prognosis (5-year-overall survival, 45%) than patients with primary cutaneous CD30– large T-cell lymphomas (12%) and patients presenting with concurrent extracutaneous disease (12%). The favorable prognosis in this group with primary cutaneous small- or medium-sized T-cell lymphomas was particularly found in patients presenting with localized skin lesions expressing a CD3+CD4+CD8– phenotype. In the primary cutaneous T-cell lymphoma (CTCL) group 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 was a group of primary cutaneous small- or medium-sized pleomorphic CTCLs with a CD3+CD4+CD8– phenotype and presenting with localized skin lesions.
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