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 Blood, 15 May 2005, Vol. 105, No. 10, pp. 3768-3785.
Prepublished online as a Blood First Edition Paper on February 3, 2005; DOI 10.1182/blood-2004-09-3502.

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REVIEW ARTICLES

WHO-EORTC classification for cutaneous lymphomas

Rein Willemze, Elaine S. Jaffe, Günter Burg, Lorenzo Cerroni, Emilio Berti, Steven H. Swerdlow, Elisabeth Ralfkiaer, Sergio Chimenti, José L. Diaz-Perez, Lyn M. Duncan, Florent Grange, Nancy Lee Harris, Werner Kempf, Helmut Kerl, Michael Kurrer, Robert Knobler, Nicola Pimpinelli, Christian Sander, Marco Santucci, Wolfram Sterry, Maarten H. Vermeer, Janine Wechsler, Sean Whittaker, and Chris J. L. M. Meijer

From the Department of Dermatology, Leiden University, Medical Center, the Netherlands; Laboratory of Pathology, National Cancer Institute, National Institutes of Health, Bethesda, MD; Department of Dermatology, University Hospital Zürich, Switzerland; Department of Dermatology, University of Graz, Austria; Department of Dermatology, University of Milan-Biococca and Ospedale Magiore, Milan, Italy; Department of Pathology, Division of Hematopathology, University of Pittsburgh School of Medicine, PA; Department of Pathology, University of Copenhagen, Denmark; Department of Dermatology, University of Rome, Italy; Department of Dermatology, Cruces Hospital, Bilbao, Spain; Department of Pathology, Massachusetts General Hospital and Harvard Medical School, Boston, MA; Department of Dermatology, Hôpital Pasteur, Colmar, France; Department of Pathology, University Hospital Zürich, Switzerland; Department of Dermatology, University of Vienna, Austria; Department of Dermatological Sciences, University of Florence, Italy; Department of Dermatology, Allgemeines Krankenhaus St Georg, Hamburg, Germany; Department of Human Pathology and Oncology, University of Florence, Italy; Department of Dermatology, Charité, Humboldt University, Berlin, Germany; Department of Pathology, Hôpital Henri Mondor, Creteil, France; Skin Tumour Unit, St John's Institute of Dermatology, St Thomas' Hospital, London, England; and Department of Pathology, Vrije Universiteit Medical Center, Amsterdam, the Netherlands.

Primary cutaneous lymphomas are currently classified by the European Organization for Research and Treatment of Cancer (EORTC) classification or the World Health Organization (WHO) classification, but both systems have shortcomings. In particular, differences in the classification of cutaneous T-cell lymphomas other than mycosis fungoides, Sézary syndrome, and the group of primary cutaneous CD30+ lymphoproliferative disorders and the classification and terminology of different types of cutaneous B-cell lymphomas have resulted in considerable debate and confusion. During recent consensus meetings representatives of both systems reached agreement on a new classification, which is now called the WHO-EORTC classification. In this paper we describe the characteristic features of the different primary cutaneous lymphomas and other hematologic neoplasms frequently presenting in the skin, and discuss differences with the previous classification schemes. In addition, the relative frequency and survival data of 1905 patients with primary cutaneous lymphomas derived from Dutch and Austrian registries for primary cutaneous lymphomas are presented to illustrate the clinical significance of this new classification.


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