Survival and Prognostic Factors in Patients with Aggressive Cutaneous T-cell Lymphomas
DOI:
https://doi.org/10.2340/actadv.v102.1087Keywords:
aggressive cutaneous T-cell lymphoma, mycosis fungoides, Sézary syndrome, prognosis, survivalAbstract
Aggressive primary cutaneous T-cell lymphomas include advanced-stage mycosis fungoides (stage ≥ IIB mycosis fungoides), Sézary syndrome, gamma/delta cutaneous lymphoma, nasal type lymphoma, aggressive epidermotropic CD8+ T-cell lymphoma and some cutaneous lymphomas not otherwise specified. To evaluate their long-term prognosis, we conducted a retrospective cohort study of 85 patients diagnosed between 2005 and 2020 with advanced-stage mycosis fungoides (n = 48), Sézary syndrome (n = 28) or aggressive non-mycosis fungoides/Sézary syndrome subtypes (n = 9). The median survival times in these 3 groups were 118.7, 45.7 and 11.2 months, respectively, and the 5-year survival rates were 55.3%, 27.8% and 33.3%, respectively. Multivariate analyses in patients with mycosis fungoides/Sézary syndrome identified age ≥ 70 years, Eastern Cooperative Oncology Group Performance Status ≥ 2, and the high-risk group according to the Cutaneous Lymphoma International Consortium prognostic model, as adverse prognostic factors. Seven patients in this mycosis fungoides/ Sézary syndrome group were in complete long-term remission after treatment with bexarotene, including 4 patients living without any treatment for 16–101 months.
Downloads
References
Willemze R, Jaffe ES, Burg G, Cerroni L, Berti E, Swerdlow SH, et al. WHO-EORTC classification for cutaneous lymphomas. Blood 2005; 105: 3768-3785.
https://doi.org/10.1182/blood-2004-09-3502 DOI: https://doi.org/10.1182/blood-2004-09-3502
Groves FD, Linet MS, Travis LB, Devesa SS. Cancer surveillance series: non-Hodgkin's lymphoma incidence by histologic subtype in the United States from 1978 through 1995. J Natl Cancer Inst 2000; 92: 1240-1251.
https://doi.org/10.1093/jnci/92.15.1240 DOI: https://doi.org/10.1093/jnci/92.15.1240
Bradford PT, Devesa SS, Anderson WF, Toro JR. Cutaneous lymphoma incidence patterns in the United States: a population-based study of 3884 cases. Blood 2009; 113: 5064-5073.
https://doi.org/10.1182/blood-2008-10-184168 DOI: https://doi.org/10.1182/blood-2008-10-184168
Olsen E, Vonderheid E, Pimpinelli N, Willemze R, Kim Y, Knobler R, 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 2007; 110: 1713-1722.
https://doi.org/10.1182/blood-2007-03-055749 DOI: https://doi.org/10.1182/blood-2007-03-055749
Mourad A, Gniadecki R. Overall survival in mycosis fungoides: a systematic review and meta-analysis. J Invest Dermatol 2020; 140: 495-497.
https://doi.org/10.1016/j.jid.2019.07.712 DOI: https://doi.org/10.1016/j.jid.2019.07.712
Scarisbrick JJ, Prince HM, Vermeer MH, Quaglino P, Horwitz S, Porcu P, et al. Cutaneous Lymphoma International Consortium Study of outcome in advanced stages of mycosis fungoides and Sézary syndrome: effect of specific prognostic markers on survival and development of a prognostic model. J Clin Oncol Off J Am Soc Clin Oncol 2015; 33: 3766-3773.
https://doi.org/10.1200/JCO.2015.61.7142 DOI: https://doi.org/10.1200/JCO.2015.61.7142
Scarisbrick JJ, Kim YH, Whittaker SJ, Wood GS, Vermeer MH, Prince HM, et al. Prognostic factors, prognostic indices and staging in mycosis fungoides and Sézary syndrome: where are we now? Br J Dermatol 2014; 170: 1226-1236.
https://doi.org/10.1111/bjd.12909 DOI: https://doi.org/10.1111/bjd.12909
Benton EC, Crichton S, Talpur R, Agar NS, Fields PA, Wedgeworth E, et al. A cutaneous lymphoma international prognostic index (CLIPi) for mycosis fungoides and Sezary syndrome. Eur J Cancer Oxf Engl 1990 2013; 49: 2859-2868.
https://doi.org/10.1016/j.ejca.2013.04.018 DOI: https://doi.org/10.1016/j.ejca.2013.04.018
Laban É, Beylot-Barry M, Ortonne N, Battistella M, Carlotti A, de Muret A, et al. Lymphoproliférations cutanées: proposition d'algorithmes diagnostiques à partir de l'expérience sur 2 ans des réseaux INCa (LYMPHOPATH et GFELC) sur 2760 lymphoproliférations cutanées. Ann Pathol 2015; 35: 131-147.
https://doi.org/10.1016/j.annpat.2015.02.001 DOI: https://doi.org/10.1016/j.annpat.2015.02.001
Grange F, Hedelin G, Joly P, Beylot-Barry M, D'Incan M, Delaunay M, et al. Prognostic factors in primary cutaneous lymphomas other than mycosis fungoides and the Sézary syndrome. The French Study Group on Cutaneous Lymphomas. Blood 1999; 93: 3637-3642.
Bouaziz J-D, Bastuji-Garin S, Poszepczynska-Guigné E, Wechsler J, Bagot M. Relative frequency and survival of patients with primary cutaneous lymphomas: data from a single-centre study of 203 patients. Br J Dermatol 2006; 154: 1206-1207.
https://doi.org/10.1111/j.1365-2133.2006.07264.x DOI: https://doi.org/10.1111/j.1365-2133.2006.07264.x
Beylot-Barry M, Vergier B, Masquelier B, Bagot M, Joly P, Souteyrand P, et al. The spectrum of cutaneous lymphomas in HIV infection: a study of 21 cases. Am J Surg Pathol 1999; 23: 1208-1216.
https://doi.org/10.1097/00000478-199910000-00005 DOI: https://doi.org/10.1097/00000478-199910000-00005
Swerdlow SH, Campo E, Pileri SA, Harris NL, Stein H, Siebert R, et al. The 2016 revision of the World Health Organization classification of lymphoid neoplasms. Blood 2016; 127: 2375-2390.
https://doi.org/10.1182/blood-2016-01-643569 DOI: https://doi.org/10.1182/blood-2016-01-643569
Kim YH, Willemze R, Pimpinelli N, Whittaker S, Olsen EA, Ranki A, et al. 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 2007; 110: 479-484.
https://doi.org/10.1182/blood-2006-10-054601 DOI: https://doi.org/10.1182/blood-2006-10-054601
Salhany KE, Cousar JB, Greer JP, Casey TT, Fields JP, Collins RD. Transformation of cutaneous T cell lymphoma to large cell lymphoma. A clinicopathologic and immunologic study. Am J Pathol 1988; 132: 265-277.
Zackheim HS, Amin S, Kashani-Sabet M, McMillan A. Prognosis in cutaneous T-cell lymphoma by skin stage: long-term survival in 489 patients. J Am Acad Dermatol 1999; 40: 418-425.
https://doi.org/10.1016/S0190-9622(99)70491-3 DOI: https://doi.org/10.1016/S0190-9622(99)70491-3
Talpur R, Singh L, Daulat S, Liu P, Seyfer S, Trynosky T, et al. Long-term outcomes of 1,263 patients with mycosis fungoides and Sézary syndrome from 1982 to 2009. Clin Cancer Res 2012; 18: 5051-5060.
https://doi.org/10.1158/1078-0432.CCR-12-0604 DOI: https://doi.org/10.1158/1078-0432.CCR-12-0604
Kim YH, Liu HL, Mraz-Gernhard S, Varghese A, Hoppe RT. Long-term outcome of 525 patients with mycosis fungoides and Sezary syndrome: clinical prognostic factors and risk for disease progression. Arch Dermatol 2003; 139: 857-866.
https://doi.org/10.1001/archderm.139.7.857 DOI: https://doi.org/10.1001/archderm.139.7.857
Nikolaou V, Papadavid E, Patsatsi A, Siakantaris M, Economidi A, Marinos L, et al. Prognostic indicators for mycosis fungoides in a Greek population. Br J Dermatol 2017; 176: 1321-1330.
https://doi.org/10.1111/bjd.15000 DOI: https://doi.org/10.1111/bjd.15000
Danish HH, Liu S, Jhaveri J, Flowers CR, Lechowicz MJ, Esiashvili N, et al. Validation of cutaneous lymphoma international prognostic index (CLIPI) for mycosis fungoides and Sézary syndrome. Leuk Lymphoma 2016; 57: 2813-2819.
https://doi.org/10.3109/10428194.2016.1173210 DOI: https://doi.org/10.3109/10428194.2016.1173210
Duvic M, Hymes K, Heald P, Breneman D, Martin AG, Myskowski P, et al. Bexarotene is effective and safe for treatment of refractory advanced-stage cutaneous T-cell lymphoma: multinational phase II-III trial results. J Clin Oncol 2001; 19: 2456-2471.
https://doi.org/10.1200/JCO.2001.19.9.2456 DOI: https://doi.org/10.1200/JCO.2001.19.9.2456
Talpur R, Ward S, Apisarnthanarax N, Breuer-Mcham J, Duvic M. Optimizing bexarotene therapy for cutaneous T-cell lymphoma. J Am Acad Dermatol 2002; 47: 672-684.
https://doi.org/10.1067/mjd.2002.124607 DOI: https://doi.org/10.1067/mjd.2002.124607
Published
How to Cite
License
Copyright (c) 2022 Joséphine Franceschi, Marine Ehret, Laetitia Visseaux, Anne Durlach, Coralie Barbe, Éric Durot, Florent Grange
![Creative Commons License](http://i.creativecommons.org/l/by-nc/4.0/88x31.png)
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
All digitalized ActaDV contents is available freely online. The Society for Publication of Acta Dermato-Venereologica owns the copyright for all material published until volume 88 (2008) and as from volume 89 (2009) the journal has been published fully Open Access, meaning the authors retain copyright to their work.
Unless otherwise specified, all Open Access articles are published under CC-BY-NC licences, allowing third parties to copy and redistribute the material in any medium or format and to remix, transform, and build upon the material for non-commercial purposes, provided proper attribution to the original work.