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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.
Previous Article | Next Article 
Submitted September 10, 2004
Accepted January 11, 2005
WHO-EORTC classification for cutaneous lymphomas
Rein Willemze*, Elaine S Jaffe, Gunter Burg, Lorenzo Cerroni, Emilio Berti, Steven H Swerdlow, Elisabeth Ralfkiaer, Sergio Chimenti, Jose L Diaz-Perez, Lyn M Duncan, Florent Grange, Nancy L 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 Meijer
Dept. of Dermatology, Leiden University Medical Center, Leiden, The Netherlands
Laboratory of Pathology, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA
Dept. of Dermatology, University Hospital Zurich, Zurich, Switzerland
Dept. of Dermatology, University of Graz, Graz, Austria
Dept. of Dermatology, University of Milan-Biococca and Ospedale Magiore, IRCCS, Milan, Italy
Dept. of Pathology, Division of Hematopathology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA
Dept. of Pathology, University of Copenhagen, Copenhagen, Denmark
Dept. of Dermatology, University of Rome, Rome, Italy
Dept. of Dermatology, Cruces Hospital, Bilbao, Spain
Dept. of Pathology, Massachusetts General Hospital and Harvard Medical School, Boston, MA, USA
Dept. of Dermatology, Hopital Pasteur, Colmar, France
Dept. of Pathology, University Hospital Zurich, Zurich, Switzerland
Dept. of Dermatology, University of Vienna, Vienna, Austria
Dept. of Dermatological Sciences, University of Florence, Florence, Italy
Dept. of Dermatology, Allgemeines Krankenhaus St. Georg, Hamburg, Germany
Dept. of Human Pathology and Oncology, University of Florence, Florence, Italy
Dept. of Dermatology, Charite, Humboldt University, Berlin, Germany
Dept. of Pathology, Hopital Henri Mondor, Creteil, France
Skin Tumour Unit, St. John's Institute of Dermatology, St. Thomas' Hospital, London, England
Dept. of Pathology, Vrije Universiteit Medical Center, Amsterdam, The Netherlands
* Corresponding author; email: rein.willemze{at}planet.nl.
Primary cutaneous lymphomas are currently classified by the EORTC classification or the WHO classification, but both systems have shortcomings. In particular, differences in the classification of cutaneous T-cell lymphomas other than mycosis fungoides, Sezary syndrome and the group of primary cutaneous CD30-positive 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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J. Shin, S. Monti, D. J. Aires, M. Duvic, T. Golub, D. A. Jones, and T. S. Kupper
Lesional gene expression profiling in cutaneous T-cell lymphoma reveals natural clusters associated with disease outcome
Blood,
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[Abstract]
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E. M. Buda-Okreglak, M. J. Walden, and M. D. Brissette
Perineural CNS Invasion in Primary Cutaneous Follicular Center Lymphoma
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P. L. Zinzani, G. Musuraca, M. Tani, V. Stefoni, E. Marchi, M. Fina, C. Pellegrini, L. Alinari, E. Derenzini, A. de Vivo, et al.
Phase II Trial of Proteasome Inhibitor Bortezomib in Patients With Relapsed or Refractory Cutaneous T-Cell Lymphoma
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D. Winter, J. Moser, E. Kriehuber, C. Wiesner, R. Knobler, F. Trautinger, P. Bombosi, G. Stingl, P. Petzelbauer, A. Rot, et al.
Down-Modulation of CXCR3 Surface Expression and Function in CD8+ T Cells from Cutaneous T Cell Lymphoma Patients
J. Immunol.,
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D. L. Longo
Watch out, MF!
Blood,
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E. Olsen, E. Vonderheid, N. Pimpinelli, R. Willemze, Y. Kim, R. Knobler, H. Zackheim, M. Duvic, T. Estrach, S. Lamberg, et al.
Revisions to the staging and classification of mycosis fungoides and Sezary syndrome: a proposal of the International Society for Cutaneous Lymphomas (ISCL) and the cutaneous lymphoma task force of the European Organization of Research and Treatment of Cancer (EORTC)
Blood,
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[Abstract]
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S. C.-S. Hu, K.-B. Tsai, G.-S. Chen, and P.-H. Chen
Infantile CD4+/CD56+ hematodermic neoplasm
Haematologica,
September 1, 2007;
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E. Callet-Bauchu, G. Salles, S. Gazzo, S. Dalle, F. Berger, and S. Hayette
Identification of a novel e8/a4 BCR/ABL fusion transcript in a case of a transformed Sezary syndrome
Haematologica,
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[Abstract]
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F. Grange, M. Beylot-Barry, P. Courville, E. Maubec, M. Bagot, B. Vergier, P. Souteyrand, L. Machet, S. Dalac, E. Esteve, et al.
Primary Cutaneous Diffuse Large B-Cell Lymphoma, Leg Type: Clinicopathologic Features and Prognostic Analysis in 60 Cases
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C. Assaf, S. Gellrich, S. Whittaker, A. Robson, L. Cerroni, C. Massone, H. Kerl, C. Rose, A. Chott, S. Chimenti, et al.
CD56-positive haematological neoplasms of the skin: a multicentre study of the Cutaneous Lymphoma Project Group of the European Organisation for Research and Treatment of Cancer
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E. A. Olsen, Y. H. Kim, T. M. Kuzel, T. R. Pacheco, F. M. Foss, S. Parker, S. R. Frankel, C. Chen, J. L. Ricker, J. M. Arduino, et al.
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Y. H. Kim, R. Willemze, N. Pimpinelli, S. Whittaker, E. A. Olsen, A. Ranki, R. Dummer, R. T. Hoppe, and for the ISCL and the EORTC
TNM classification system for primary cutaneous lymphomas other than mycosis fungoides and Sezary syndrome: a proposal of the International Society for Cutaneous Lymphomas (ISCL) and the Cutaneous Lymphoma Task Force of the European Organization of Research and Treatment of Cancer (EORTC)
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D. Sanchez-Cano, J. Callejas-Rubio, A. Vilanova-Mateu, M. Gomez-Morales, and N. Ortego-Centeno
Intravascular lymphoma in a patient with systemic lupus erythematosus: a case report
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[Abstract]
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S. R. Lessin
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J. Guitart and C. Magro
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M. G. Bernengo, P. Quaglino, A. Comessatti, M. Ortoncelli, M. Novelli, F. Lisa, and M. T. Fierro
Low-dose intermittent alemtuzumab in the treatment of Sezary syndrome: clinical and immunologic findings in 14 patients
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P. Garrido, F. Ruiz-Cabello, P. Barcena, Y. Sandberg, J. Canton, M. Lima, A. Balanzategui, M. Gonzalez, M. A. Lopez-Nevot, A. W. Langerak, et al.
Monoclonal TCR-V 13.1+/CD4+/NKa+/CD8 /+dim T-LGL lymphocytosis: evidence for an antigen-driven chronic T-cell stimulation origin
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Y. H. Kim, M. Duvic, E. Obitz, R. Gniadecki, L. Iversen, A. Osterborg, S. Whittaker, T. M. Illidge, T. Schwarz, R. Kaufmann, et al.
Clinical efficacy of zanolimumab (HuMax-CD4): two phase 2 studies in refractory cutaneous T-cell lymphoma
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S. Pulini, S. Rupoli, G. Goteri, N. Pimpinelli, R. Alterini, A. Tassetti, A. R. Scortechini, M. Offidani, S. Mulattieri, A. Stronati, et al.
Pegylated liposomal doxorubicin in the treatment of primary cutaneous T-cell lymphomas
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N. J. Senff, J. J. Hoefnagel, P. M. Jansen, M. H. Vermeer, J. van Baarlen, W. A. Blokx, M. R. Canninga-van Dijk, M.-L. Geerts, K. M. Hebeda, P. M. Kluin, et al.
Reclassification of 300 Primary Cutaneous B-Cell Lymphomas According to the New WHO-EORTC Classification for Cutaneous Lymphomas: Comparison With Previous Classifications and Identification of Prognostic Markers
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A. Woetmann, P. Lovato, K. W. Eriksen, T. Krejsgaard, T. Labuda, Q. Zhang, A.-M. Mathiesen, C. Geisler, A. Svejgaard, M. A. Wasik, et al.
Nonmalignant T cells stimulate growth of T-cell lymphoma cells in the presence of bacterial toxins
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C. M Bacon, M.-Q. Du, and A. Dogan
Mucosa-associated lymphoid tissue (MALT) lymphoma: a practical guide for pathologists
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A Nodule on a Boy's Back--Diagnosis
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Persistent Violaceous Papules on the Ears--Diagnosis
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R. Dijkman, R. van Doorn, K. Szuhai, R. Willemze, M. H. Vermeer, and C. P. Tensen
Gene-expression profiling and array-based CGH classify CD4+CD56+ hematodermic neoplasm and cutaneous myelomonocytic leukemia as distinct disease entities
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G. A. Salles
Clinical Features, Prognosis and Treatment of Follicular Lymphoma
Hematology,
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M. Duvic, R. Talpur, X. Ni, C. Zhang, P. Hazarika, C. Kelly, J. H. Chiao, J. F. Reilly, J. L. Ricker, V. M. Richon, et al.
Phase 2 trial of oral vorinostat (suberoylanilide hydroxamic acid, SAHA) for refractory cutaneous T-cell lymphoma (CTCL)
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R. Bergman, Z. Khamaysi, D. Sahar, and Y. Ben-Arieh
Cutaneous Lymphoid Hyperplasia Presenting as a Solitary Facial Nodule: Clinical, Histopathological, Immunophenotypical, and Molecular Studies
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D Errante, D Bernardi, A Bianco, S De Nardi, and L Salvagno
Rituximab-related urticarial reaction in a patient treated for primary cutaneous B-cell lymphoma
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M. Bouzani, T. Karmiris, D. Rontogianni, S. Delimpassi, J. Apostolidis, M. Mpakiri, and E. Nikiforakis
Disseminated Intravascular B-Cell Lymphoma: Clinicopathological Features and Outcome of Three Cases Treated with Anthracycline-Based Immunochemotherapy
Oncologist,
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923 - 928.
[Abstract]
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R. Gruber, N. T. Sepp, P. O. Fritsch, and M. Schmuth
Prognosis of Lymphomatoid Papulosis
Oncologist,
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J. R. Brown
In Reply
Oncologist,
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P. L. Zinzani, P. Quaglino, N. Pimpinelli, E. Berti, G. Baliva, S. Rupoli, M. Martelli, M. Alaibac, G. Borroni, S. Chimenti, et al.
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S. Hahtola, S. Tuomela, L. Elo, T. Hakkinen, L. Karenko, B. Nedoszytko, H. Heikkila, U. Saarialho-Kere, J. Roszkiewicz, T. Aittokallio, et al.
Th1 Response and Cytotoxicity Genes Are Down-Regulated in Cutaneous T-Cell Lymphoma.
Clin. Cancer Res.,
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L Cerroni
Lymphoproliferative lesions of the skin.
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E. Jacobsen
Anaplastic Large-Cell Lymphoma, T-/Null-Cell Type
Oncologist,
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[Abstract]
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Reviews of Note
Journal Watch Dermatology,
June 27, 2006;
2006(627):
9 - 9.
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R. Dijkman, C. P. Tensen, M. Buettner, G. Niedobitek, R. Willemze, and M. H. Vermeer
Primary cutaneous follicle center lymphoma and primary cutaneous large B-cell lymphoma, leg type, are both targeted by aberrant somatic hypermutation but demonstrate differential expression of AID
Blood,
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4926 - 4929.
[Abstract]
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Y. Fujita, R. Abe, M. Sasaki, A. Honda, M. Furuichi, Y. Asano, O. Norisugi, T. Shimizu, and H. Shimizu
Presence of Circulating CCR10+ T cells and Elevated Serum CTACK/CCL27 in the Early Stage of Mycosis Fungoides.
Clin. Cancer Res.,
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E. Zambrano, O. Mejia-Mejia, C. Bifulco, J. Shin, and M. Reyes-Mugica
Extranodal Marginal Zone B-Cell Lymphoma/Maltoma of the Lip in a Child: Case Report and Review of Cutaneous Lymphoid Proliferations in Childhood
International Journal of Surgical Pathology,
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P. L. Zinzani, P. Quaglino, N. Pimpinelli, E. Berti, G. Baliva, S. Rupoli, M. Martelli, M. Alaibac, G. Borroni, S. Chimenti, et al.
Prognostic Factors in Primary Cutaneous B-Cell Lymphoma: The Italian Study Group for Cutaneous Lymphomas
J. Clin. Oncol.,
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