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EORTC Classification for Primary Cutaneous Lymphomas: A Proposal From the Cutaneous Lymphoma Study Group of the European Organization for Research and Treatment of Cancer
R. Willemze,
H. Kerl,
W. Sterry,
E. Berti,
L. Cerroni,
S. Chimenti,
J.L. Diaz-Peréz,
M.L. Geerts,
M. Goos,
R. Knobler,
E. Ralfkiaer,
M. Santucci,
N. Smith,
J. Wechsler,
W.A. van Vloten, and
C.J.L.M. Meijer
From the Departments of Dermatology and Pathology, Free University Hospital, Amsterdam, The Netherlands; the Department of Dermatology, University of Graz, Austria; the Department of Dermatology, Charité, Humboldt University, Berlin, Germany; the Department of Dermatology, University of Milan, Italy; the Department of Dermatology, University of L'Aquila, Italy; the Department of Dermatology, Cruces Hospital, Bilbao, Spain; the Department of Dermatology, University of Gent, Belgium; the Department of Dermatology, University of Essen, Germany; the Department of Dermatology, University of Vienna, Austria; the Department of Pathology, Herlev Hospital, University of Copenhagen, Denmark; the Department of Pathology, University of Florence, Italy; the Department of Dermatology, St John's Hospital, London, UK; the Department of Pathology, Hospital Henri Mondor, Paris, France; the Department of Dermatology, University of Utrecht, The Netherlands.
Primary cutaneous lymphomas represent a heterogeneous group of T- and B-cell lymphomas that show considerable variation in histology, phenotype, and prognosis. Recently, the European Organization for Research and Treatment of Cancer (EORTC) Cutaneous Lymphoma Project Group has reached consensus on a new classification for this group of diseases. The EORTC classification for primary cutaneous lymphomas is based on a combination of clinical, histologic, and immunophenotypic criteria, and thus contains well-defined disease entities rather than histologic subgroups. In addition, this new classification contains a number of provisional entities, which may display characteristic histologic features, but are not yet well defined clinically. These provisional entities account for less than 5% of all primary cutaneous lymphomas. In this report the basic principles of this new classification, as well as the characteristic features of the different disease entities, are described. In addition, survival data of 626 patients with primary cutaneous lymphomas derived from the registry of the Dutch Cutaneous Lymphoma Working Group, illustrating the clinical validity of this new classification, are presented.
Blood, Vol. 90 No. 1 (July 1), 1997:
pp. 354-371
© 1997 by The American Society of Hematology.

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Primary cutaneous non-Hodgkin's lymphoma with aggressive histology: inferior outcome is associated with peripheral T-cell type and elevated lactate dehydrogenase, but not extent of cutaneous involvement
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August 1, 2002;
13(8):
1290 - 1299.
[Abstract]
[Full Text]
[PDF]
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M. Nikolova, P. Musette, M. Bagot, L. Boumsell, and A. Bensussan
Engagement of ILT2/CD85j in Sezary syndrome cells inhibits their CD3/TCR signaling
Blood,
July 18, 2002;
100(3):
1019 - 1025.
[Abstract]
[Full Text]
[PDF]
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M. Steinhoff, M. Hummel, I. Anagnostopoulos, P. Kaudewitz, V. Seitz, C. Assaf, C. Sander, and H. Stein
Single-cell analysis of CD30+ cells in lymphomatoid papulosis demonstrates a common clonal T-cell origin
Blood,
June 28, 2002;
100(2):
578 - 584.
[Abstract]
[Full Text]
[PDF]
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R Goyal, R J Motley, S D Dojcinov, and C M Lane
Cutaneous presentation of orbital follicular lymphoma: clinical differential diagnosis with lymphocytoma cutis
Br J Ophthalmol,
May 1, 2002;
86(5):
596 - 597.
[Full Text]
[PDF]
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D. Breneman, M. Duvic, T. Kuzel, R. Yocum, J. Truglia, and V. J. Stevens
Phase 1 and 2 Trial of Bexarotene Gel for Skin-Directed Treatment of Patients With Cutaneous T-Cell Lymphoma
Arch Dermatol,
March 1, 2002;
138(3):
325 - 332.
[Abstract]
[Full Text]
[PDF]
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I. Mirza, N. Macpherson, S. Paproski, R. D. Gascoyne, B. Yang, W. G. Finn, and E. D. Hsi
Primary Cutaneous Follicular Lymphoma: An Assessment of Clinical, Histopathologic, Immunophenotypic, and Molecular Features
J. Clin. Oncol.,
February 1, 2002;
20(3):
647 - 655.
[Abstract]
[Full Text]
[PDF]
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L. Cerroni, R. Fink-Puches, B. Back, and H. Kerl
Follicular Mucinosis: A Critical Reappraisal of Clinicopathologic Features and Association With Mycosis Fungoides and Sezary Syndrome
Arch Dermatol,
February 1, 2002;
138(2):
182 - 189.
[Abstract]
[Full Text]
[PDF]
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R. van Doorn, E. Scheffer, R. Willemze, and for the Dutch Cutaneous Lymphoma Group
Follicular Mycosis Fungoides, a Distinct Disease Entity With or Without Associated Follicular Mucinosis: A Clinicopathologic and Follow-up Study of 51 Patients
Arch Dermatol,
February 1, 2002;
138(2):
191 - 198.
[Abstract]
[Full Text]
[PDF]
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R. Dummer, J. Kamarashev, W. Kempf, A. C. Haffner, M. Hess-Schmid, and G. Burg
Junctional CD8+ Cutaneous Lymphomas With Nonaggressive Clinical Behavior: A CD8+ Variant of Mycosis Fungoides?
Arch Dermatol,
February 1, 2002;
138(2):
199 - 203.
[Abstract]
[Full Text]
[PDF]
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R. Fink-Puches, P. Zenahlik, B. Back, J. Smolle, H. Kerl, and L. Cerroni
Primary cutaneous lymphomas: applicability of current classification schemes (European Organization for Research and Treatment of Cancer, World Health Organization) based on clinicopathologic features observed in a large group of patients
Blood,
February 1, 2002;
99(3):
800 - 805.
[Abstract]
[Full Text]
[PDF]
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M. Urosevic, J. Willers, B. Mueller, W. Kempf, G. Burg, and R. Dummer
HLA-G protein up-regulation in primary cutaneous lymphomas is associated with interleukin-10 expression in large cell T-cell lymphomas and indolent B-cell lymphomas
Blood,
January 15, 2002;
99(2):
609 - 617.
[Abstract]
[Full Text]
[PDF]
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J. M. Connors, E. D. Hsi, and F. M. Foss
Lymphoma of the Skin
Hematology,
January 1, 2002;
2002(1):
263 - 282.
[Abstract]
[Full Text]
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M. H. Vermeer, R. van Doorn, D. Dukers, M. W. Bekkenk, C. J.L.M. Meijer, and R. Willemze
CD8+ T Cells in Cutaneous T-Cell Lymphoma: Expression of Cytotoxic Proteins, Fas Ligand, and Killing Inhibitory Receptors and Their Relationship With Clinical Behavior
J. Clin. Oncol.,
December 1, 2001;
19(23):
4322 - 4329.
[Abstract]
[Full Text]
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N. V. Kamath, A. C. Gilliam, M. Nihal, T. P. Spiro, and G. S. Wood
Primary Cutaneous Large B-Cell Lymphoma of the Leg Relapsing as Cutaneous Intravascular Large B-Cell Lymphoma
Arch Dermatol,
December 1, 2001;
137(12):
1657 - 1658.
[Full Text]
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K. D. Berg, N. K. Brinster, K. M. Huhn, M. G. Goggins, R. J. Jones, A. Makary, K. M. Murphy, C. A. Griffin, L. S. Rosenblum-Vos, M. J. Borowitz, et al.
Transmission of a T-Cell Lymphoma by Allogeneic Bone Marrow Transplantation
N. Engl. J. Med.,
November 15, 2001;
345(20):
1458 - 1463.
[Full Text]
[PDF]
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G. S. Wood
T-Cell Receptor and Immunoglobulin Gene Rearrangements in Diagnosing Skin Disease
Arch Dermatol,
November 1, 2001;
137(11):
1503 - 1506.
[Full Text]
[PDF]
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R. L. Piekarz, R. Robey, V. Sandor, S. Bakke, W. H. Wilson, L. Dahmoush, D. M. Kingma, M. L. Turner, R. Altemus, and S. E. Bates
Inhibitor of histone deacetylation, depsipeptide (FR901228), in the treatment of peripheral and cutaneous T-cell lymphoma: a case report
Blood,
November 1, 2001;
98(9):
2865 - 2868.
[Abstract]
[Full Text]
[PDF]
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F. Grange, M. W. Bekkenk, J. Wechsler, C. J.L.M. Meijer, L. Cerroni, M. Bernengo, J. Bosq, G. Hedelin, R. Fink Puches, W. A. van Vloten, et al.
Prognostic Factors in Primary Cutaneous Large B-Cell Lymphomas: A European Multicenter Study
J. Clin. Oncol.,
August 15, 2001;
19(16):
3602 - 3610.
[Abstract]
[Full Text]
[PDF]
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M. H. Vermeer, C. P. Tensen, P. M. van der Stoop, H. W. van Oostveen, M. Lund, R. J. Scheper, and R. Willemze
Absence of TH2 Cytokine Messenger RNA Expression in CD30-Negative Primary Cutaneous Large T-Cell Lymphomas
Arch Dermatol,
July 1, 2001;
137(7):
901 - 905.
[Abstract]
[Full Text]
[PDF]
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P. Rubegni, G. De Aloe, M. Di Renzo, G. Pompella, A. L. Pasqui, A. Auteri, L. Andreassi, and M. Fimiani
Cytokine Production Profile of Peripheral Blood Mononuclear Cells in Patients With Large-Plaque Parapsoriasis
Arch Dermatol,
July 1, 2001;
137(7):
966 - 967.
[Full Text]
[PDF]
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J. Kamarashev, G. Burg, M. C. Mingari, W. Kempf, G. Hofbauer, and R. Dummer
Differential Expression of Cytotoxic Molecules and Killer Cell Inhibitory Receptors in CD8+ and CD56+ Cutaneous Lymphomas
Am. J. Pathol.,
May 1, 2001;
158(5):
1593 - 1598.
[Abstract]
[Full Text]
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R. Bergman, P. J. Kurtin, L. E. Gibson, P. R. Hull, T. K. Kimlinger, and A. L. Schroeter
Clinicopathologic, Immunophenotypic, and Molecular Characterization of Primary Cutaneous Follicular B-Cell Lymphoma
Arch Dermatol,
April 1, 2001;
137(4):
432 - 439.
[Abstract]
[Full Text]
[PDF]
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L. Cerroni and H. Kerl
Primary Cutaneous Follicular Lymphoma
Arch Dermatol,
April 1, 2001;
137(4):
489 - 490.
[Full Text]
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D F Dukers, M H Vermeer, L H Jaspars, C A Sander, M J Flaig, W Vos, R Willemze, and C J L M Meijer
Expression of killer cell inhibitory receptors is restricted to true NK cell lymphomas and a subset of intestinal enteropathy-type T cell lymphomas with a cytotoxic phenotype
J. Clin. Pathol.,
March 1, 2001;
54(3):
224 - 228.
[Abstract]
[Full Text]
[PDF]
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M. Bagot, A. Moretta, S. Sivori, R. Biassoni, C. Cantoni, C. Bottino, L. Boumsell, and A. Bensussan
CD4+ cutaneous T-cell lymphoma cells express the p140-killer cell immunoglobulin-like receptor
Blood,
March 1, 2001;
97(5):
1388 - 1391.
[Abstract]
[Full Text]
[PDF]
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T. E. Hansen-Hagge, U. Trefzer, A. S. zu Reventlow, K. Kaltoft, and W. Sterry
Identification of sample-specific sequences in mammalian cDNA and genomic DNA by the novel ligation-mediated subtraction (Limes)
Nucleic Acids Res.,
February 15, 2001;
29(4):
e20 - e20.
[Abstract]
[Full Text]
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J. J. Scarisbrick, S. Whittaker, A. V. Evans, E. A. Fraser-Andrews, F. J. Child, A. Dean, and R. Russell-Jones
Prognostic significance of tumor burden in the blood of patients with erythrodermic primary cutaneous T-cell lymphoma
Blood,
February 1, 2001;
97(3):
624 - 630.
[Abstract]
[Full Text]
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A. H. Sarris, I. Braunschweig, L. J. Medeiros, M. Duvic, C. S. Ha, M. A. Rodriguez, F. B. Hagemeister, P. McLaughlin, J. Romaguera, J. Cox, et al.
Primary Cutaneous Non-Hodgkin's Lymphoma of Ann Arbor Stage I: Preferential Cutaneous Relapses but High Cure Rate With Doxorubicin-Based Therapy
J. Clin. Oncol.,
January 15, 2001;
19(2):
398 - 405.
[Abstract]
[Full Text]
[PDF]
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D. Slater
Histopathological aspects of cutaneous lymphoma
J R Soc Med,
January 7, 2001;
94(7):
337 - 340.
[Full Text]
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S. Eichmüller, D. Usener, R. Dummer, A. Stein, D. Thiel, and D. Schadendorf
Serological detection of cutaneous T-cell lymphoma-associated antigens
PNAS,
January 5, 2001;
(2001)
21386498.
[Abstract]
[Full Text]
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M. Storz, K. Zepter, J. Kamarashev, R. Dummer, G. Burg, and A. C. Häffner
Coexpression of CD40 and CD40 Ligand in Cutaneous T-Cell Lymphoma (Mycosis Fungoides)
Cancer Res.,
January 1, 2001;
61(2):
452 - 454.
[Abstract]
[Full Text]
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J. P. Greer, M. C. Kinney, and T. P. Loughran Jr.
T Cell and NK Cell Lymphoproliferative Disorders
Hematology,
January 1, 2001;
2001(1):
259 - 281.
[Abstract]
[Full Text]
[PDF]
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H. Stein, H.-D. Foss, H. Durkop, T. Marafioti, G. Delsol, K. Pulford, S. Pileri, and B. Falini
CD30+ anaplastic large cell lymphoma: a review of its histopathologic, genetic, and clinical features
Blood,
December 1, 2000;
96(12):
3681 - 3695.
[Abstract]
[Full Text]
[PDF]
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M.-H. Delfau-Larue, L. Laroche, J. Wechsler, E. Lepage, C. Lahet, M. Asso-Bonnet, M. Bagot, and J.-P. Farcet
Diagnostic value of dominant T-cell clones in peripheral blood in 363 patients presenting consecutively with a clinical suspicion of cutaneous lymphoma
Blood,
November 1, 2000;
96(9):
2987 - 2992.
[Abstract]
[Full Text]
[PDF]
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R. S. Siegel, T. Pandolfino, J. Guitart, S. Rosen, and T. M. Kuzel
Primary Cutaneous T-Cell Lymphoma: Review and Current Concepts
J. Clin. Oncol.,
August 15, 2000;
18(15):
2908 - 2925.
[Abstract]
[Full Text]
[PDF]
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J. R. Toro, M. Beaty, L. Sorbara, M. L. Turner, J. White, D. W. Kingma, M. Raffeld, and E. S. Jaffe
{gamma}{delta} T-Cell Lymphoma of the Skin: A Clinical, Microscopic, and Molecular Study
Arch Dermatol,
August 1, 2000;
136(8):
1024 - 1032.
[Abstract]
[Full Text]
[PDF]
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M. E. Kadin
Cutaneous {gamma}{delta} T-Cell Lymphomas--How and Why Should They Be Recognized?
Arch Dermatol,
August 1, 2000;
136(8):
1052 - 1054.
[Full Text]
[PDF]
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E. Poszepczynska, M. Bagot, H. Echchakir, D. Martinvalet, M. Ramez, D. Charue, L. Boumsell, and A. Bensussan
Functional characterization of an IL-7-dependent CD4+CD8alpha alpha + Th3-type malignant cell line derived from a patient with a cutaneous T-cell lymphoma
Blood,
August 1, 2000;
96(3):
1056 - 1063.
[Abstract]
[Full Text]
[PDF]
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M. W. Bekkenk, F. A. M. J. Geelen, P. C. v. V. Vader, F. Heule, M.-L. Geerts, W. A. van Vloten, C. J. L. M. Meijer, and R. Willemze
Primary and secondary cutaneous CD30+ lymphoproliferative disorders: a report from the Dutch Cutaneous Lymphoma Group on the long-term follow-up data of 219 patients and guidelines for diagnosis and treatment
Blood,
June 15, 2000;
95(12):
3653 - 3661.
[Abstract]
[Full Text]
[PDF]
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L. Cerroni, E. Arzberger, B. Putz, G. Hofler, D. Metze, C. A. Sander, C. Rose, P. Wolf, A. Rutten, J. M. McNiff, et al.
Primary cutaneous follicle center cell lymphoma with follicular growth pattern
Blood,
June 15, 2000;
95(12):
3922 - 3928.
[Abstract]
[Full Text]
[PDF]
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R. L t. Berge, J. J Oudejans, G.-J. Ossenkoppele, K. Pulford, R. Willemze, B. Falini, A. Chott, and C. J L M Meijer
ALK expression in extranodal anaplastic large cell lymphoma favours systemic disease with (primary) nodal involvement and a good prognosis and occurs before dissemination
J. Clin. Pathol.,
June 1, 2000;
53(6):
445 - 450.
[Abstract]
[Full Text]
[PDF]
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T. L. Pandolfino, R. S. Siegel, T. M. Kuzel, S. T. Rosen, and J. Guitart
Primary Cutaneous B-Cell Lymphoma: Review and Current Concepts
J. Clin. Oncol.,
May 10, 2000;
18(10):
2152 - 2168.
[Abstract]
[Full Text]
[PDF]
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R. van Doorn, C. W. Van Haselen, P. C. van Voorst Vader, M.-L. Geerts, F. Heule, M. de Rie, P. M. Steijlen, S. K. Dekker, W. A. van Vloten, and R. Willemze
Mycosis Fungoides: Disease Evolution and Prognosis of 309 Dutch Patients
Arch Dermatol,
April 1, 2000;
136(4):
504 - 510.
[Abstract]
[Full Text]
[PDF]
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M. Ming and P. E. LeBoit
Can Dermatopathologists Reliably Make the Diagnosis of Mycosis Fungoides?: If Not, Who Can?
Arch Dermatol,
April 1, 2000;
136(4):
543 - 546.
[Full Text]
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J. Guitart, F. Roufosse, L. Schandene, E. Cogan, R. Suzuki, M. Seto, S. Nakaura, H.-U. Simon, and R. Dummer
Idiopathic Eosinophilia
N. Engl. J. Med.,
March 2, 2000;
342(9):
659 - 661.
[Full Text]
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