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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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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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D. L. Longo
Watch out, MF!
Blood, September 15, 2007; 110(6): 1708 - 1709.
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BloodHome page
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, September 15, 2007; 110(6): 1713 - 1722.
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haematolHome page
S. C.-S. Hu, K.-B. Tsai, G.-S. Chen, and P.-H. Chen
Infantile CD4+/CD56+ hematodermic neoplasm
Haematologica, September 1, 2007; 92(9): e91 - e93.
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haematolHome page
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, September 1, 2007; 92(9): 1277 - 1278.
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Arch DermatolHome page
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
Arch Dermatol, September 1, 2007; 143(9): 1144 - 1150.
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J. Clin. Pathol.Home page
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
J. Clin. Pathol., September 1, 2007; 60(9): 981 - 989.
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JCOHome page
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.
Phase IIB Multicenter Trial of Vorinostat in Patients With Persistent, Progressive, or Treatment Refractory Cutaneous T-Cell Lymphoma
J. Clin. Oncol., July 20, 2007; 25(21): 3109 - 3115.
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BloodHome page
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)
Blood, July 15, 2007; 110(2): 479 - 484.
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LupusHome page
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
Lupus, July 1, 2007; 16(7): 525 - 528.
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Arch DermatolHome page
S. R. Lessin
Cutaneous T-Cell Lymphoma Epidemiology: Patients Providing the Power
Arch Dermatol, July 1, 2007; 143(7): 916 - 918.
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Arch DermatolHome page
J. Guitart and C. Magro
Cutaneous T-Cell Lymphoid Dyscrasia: A Unifying Term for Idiopathic Chronic Dermatoses With Persistent T-Cell Clones
Arch Dermatol, July 1, 2007; 143(7): 921 - 932.
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haematolHome page
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
Haematologica, June 1, 2007; 92(6): 784 - 794.
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BloodHome page
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
Blood, June 1, 2007; 109(11): 4890 - 4898.
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BloodHome page
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
Blood, June 1, 2007; 109(11): 4655 - 4662.
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haematolHome page
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
Haematologica, May 1, 2007; 92(5): 686 - 689.
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JCOHome page
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
J. Clin. Oncol., April 20, 2007; 25(12): 1581 - 1587.
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BloodHome page
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
Blood, April 15, 2007; 109(8): 3325 - 3332.
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J. Clin. Pathol.Home page
C. M Bacon, M.-Q. Du, and A. Dogan
Mucosa-associated lymphoid tissue (MALT) lymphoma: a practical guide for pathologists
J. Clin. Pathol., April 1, 2007; 60(4): 361 - 372.
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Arch DermatolHome page
A Nodule on a Boy's Back--Diagnosis
Arch Dermatol, March 1, 2007; 143(3): 417 - 422.
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Arch DermatolHome page
Persistent Violaceous Papules on the Ears--Diagnosis
Arch Dermatol, March 1, 2007; 143(3): 417 - 422.
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BloodHome page
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
Blood, February 15, 2007; 109(4): 1720 - 1727.
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ASH Education BookHome page
G. A. Salles
Clinical Features, Prognosis and Treatment of Follicular Lymphoma
Hematology, January 1, 2007; 2007(1): 216 - 225.
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BloodHome page
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)
Blood, January 1, 2007; 109(1): 31 - 39.
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Arch DermatolHome page
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
Arch Dermatol, December 1, 2006; 142(12): 1561 - 1566.
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Ann OncolHome page
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
Ann. Onc., November 1, 2006; 17(11): 1720 - 1721.
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The OncologistHome page
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, September 1, 2006; 11(8): 923 - 928.
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The OncologistHome page
R. Gruber, N. T. Sepp, P. O. Fritsch, and M. Schmuth
Prognosis of Lymphomatoid Papulosis
Oncologist, September 1, 2006; 11(8): 955 - 957.
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The OncologistHome page
J. R. Brown
In Reply
Oncologist, September 1, 2006; 11(8): 957 - 957.
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JCOHome page
P. L. Zinzani, P. Quaglino, N. Pimpinelli, E. Berti, G. Baliva, S. Rupoli, M. Martelli, M. Alaibac, G. Borroni, S. Chimenti, et al.
In Reply
J. Clin. Oncol., August 20, 2006; 24(24): 4041a - 4042.
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Clin. Cancer Res.Home page
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., August 15, 2006; 12(16): 4812 - 4821.
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J. Clin. Pathol.Home page
L Cerroni
Lymphoproliferative lesions of the skin.
J. Clin. Pathol., August 1, 2006; 59(8): 813 - 826.
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The OncologistHome page
E. Jacobsen
Anaplastic Large-Cell Lymphoma, T-/Null-Cell Type
Oncologist, July 1, 2006; 11(7): 831 - 840.
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Journal Watch DermatologyHome page
Reviews of Note
Journal Watch Dermatology, June 27, 2006; 2006(627): 9 - 9.
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BloodHome page
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, June 15, 2006; 107(12): 4926 - 4929.
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Clin. Cancer Res.Home page
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., May 1, 2006; 12(9): 2670 - 2675.
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INT J SURG PATHOLHome page
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, April 1, 2006; 14(2): 163 - 169.
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JCOHome page
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., March 20, 2006; 24(9): 1376 - 1382.
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