TY - JOUR
T1 - European Organisation for Research and Treatment of Cancer, United States Cutaneous Lymphoma Consortium and International Society for Cutaneous Lymphomas consensus recommendations for management and treatment of cutaneous lymphoproliferative disorders
AU - Willemze, Rein
AU - Assaf, Chalid
AU - Bagot, Martine
AU - Beylot-Barry, Marie
AU - Berti, Emilio
AU - Busschots, Anne Marie
AU - Cerroni, Lorenzo
AU - Climent, Fina
AU - Diercks, Gilles
AU - Geskin, Larisa
AU - Gniadecki, Robert
AU - Gru, Alejandro A
AU - Guenova, Emmanuella
AU - Guitart, Joan
AU - Jansen, Patty
AU - Kempf, Werner
AU - Kim, Ellen
AU - Kim, Youn H
AU - Koldijk, Marjolein
AU - Marschalkó, Martá
AU - Mitteldorf, Christina
AU - Molgó, Montserrat
AU - Muniesa, Cristina
AU - Neelis, Karen J
AU - Olsen, Elise
AU - Ortiz-Romero, Pablo L
AU - Papadavid, Evangelia
AU - Pimpinelli, Nicola
AU - Pulitzer, Melissa
AU - Quaglino, Pietro
AU - Querfeld, Christiane
AU - Quint, Koen
AU - Scarisbrick, Julia J
AU - Schrader, Anne M R
AU - Stadler, Rudolf
AU - Vermeer, Maarten
AU - Wehkamp, Ulrike
AU - Whittaker, Sean
AU - Wobser, Marion
N1 - © The Author(s) 2025. Published by Oxford University Press on behalf of British Association of Dermatologists. All rights reserved. For commercial re-use, please contact [email protected] for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site—for further information please contact [email protected].
PY - 2025/11/18
Y1 - 2025/11/18
N2 - BACKGROUND: In recent classifications several cutaneous lymphomas were reclassified as lymphoproliferative disorder (LPD). These include primary cutaneous CD4-positive small/medium T-cell LPD (PCSM-TCLPD), primary cutaneous acral CD8+ T-cell LPD (acral CD8+ TLPD) and primary cutaneous marginal zone LPD (PCMZLPD; still classified as primary cutaneous marginal zone lymphoma (PCMZL) in the 5th edition of the WHO classification). The results of a survey among 30 cutaneous lymphoma centres on the effects of this new terminology on clinical management revealed considerable heterogeneity and emphasized the need to develop uniform recommendations for management and treatment of these disorders.OBJECTIVES: To develop consensus recommendations for staging, treatment and follow-up policy in PCSM-TCLPD, acral CD8+ TLPD and PCMZL/LPD.METHODS: Two surveys with questions regarding staging, treatment and follow-up strategy of cutaneous LPDs were distributed among 30 cutaneous lymphoma expert centres collaborating within the EORTC-CLTG, USCLC and ISCL. Based on the results of these surveys, an extensive literature search, two rounds of feedback and a final consensus meeting consensus recommendations were formulated.RESULTS: Important changes compared to current practise and literature include: staging examinations apart from thorough clinical examination of skin and peripheral lymph nodes not required in typical cases of PCSM-TCLPD and acral CD8+ TLPD; low-dose radiotherapy (4-8 Gy) rather than dose ≥20 Gy, for PCSM-TCLPD and acral CD8+ TLPD, and 4 Gy for PCMZL/LPD, with possibility to escalate to a higher dose (20-24 Gy) in case of local failure; increase in the use of intralesional corticosteroids as initial treatment in all three LPDs; limited follow-up period (2 years) in PCSM-TCLPD and acral CD8+ TLPD LPD.CONCLUSION: These EORTC/USCLC/ISCL consensus recommendations reflect the state-of-the-art management and treatment as agreed upon by major cutaneous lymphoma centers. They may contribute to uniform staging, treatment and follow-up policy in patients with cutaneous LPDs.
AB - BACKGROUND: In recent classifications several cutaneous lymphomas were reclassified as lymphoproliferative disorder (LPD). These include primary cutaneous CD4-positive small/medium T-cell LPD (PCSM-TCLPD), primary cutaneous acral CD8+ T-cell LPD (acral CD8+ TLPD) and primary cutaneous marginal zone LPD (PCMZLPD; still classified as primary cutaneous marginal zone lymphoma (PCMZL) in the 5th edition of the WHO classification). The results of a survey among 30 cutaneous lymphoma centres on the effects of this new terminology on clinical management revealed considerable heterogeneity and emphasized the need to develop uniform recommendations for management and treatment of these disorders.OBJECTIVES: To develop consensus recommendations for staging, treatment and follow-up policy in PCSM-TCLPD, acral CD8+ TLPD and PCMZL/LPD.METHODS: Two surveys with questions regarding staging, treatment and follow-up strategy of cutaneous LPDs were distributed among 30 cutaneous lymphoma expert centres collaborating within the EORTC-CLTG, USCLC and ISCL. Based on the results of these surveys, an extensive literature search, two rounds of feedback and a final consensus meeting consensus recommendations were formulated.RESULTS: Important changes compared to current practise and literature include: staging examinations apart from thorough clinical examination of skin and peripheral lymph nodes not required in typical cases of PCSM-TCLPD and acral CD8+ TLPD; low-dose radiotherapy (4-8 Gy) rather than dose ≥20 Gy, for PCSM-TCLPD and acral CD8+ TLPD, and 4 Gy for PCMZL/LPD, with possibility to escalate to a higher dose (20-24 Gy) in case of local failure; increase in the use of intralesional corticosteroids as initial treatment in all three LPDs; limited follow-up period (2 years) in PCSM-TCLPD and acral CD8+ TLPD LPD.CONCLUSION: These EORTC/USCLC/ISCL consensus recommendations reflect the state-of-the-art management and treatment as agreed upon by major cutaneous lymphoma centers. They may contribute to uniform staging, treatment and follow-up policy in patients with cutaneous LPDs.
UR - https://www.scopus.com/pages/publications/105022289116
U2 - 10.1093/bjd/ljaf312
DO - 10.1093/bjd/ljaf312
M3 - Article
C2 - 40795052
SN - 1365-2133
VL - 193
SP - 1090
EP - 1100
JO - British Journal of Dermatology
JF - British Journal of Dermatology
IS - 6
ER -