The incidence of cutaneous T-cell lymphomas (CTCL) has been reported as <10 per 100,000 population globally.[3]Dummer R, Vermeer MH, Scarisbrick JJ, et al. Cutaneous T cell lymphoma. Nat Rev Dis Primers. 2021 Aug 26;7(1):61.
http://www.ncbi.nlm.nih.gov/pubmed/34446710?tool=bestpractice.com
However, as most are low-grade malignancies with long-term survival, the prevalence of CTCL is up to 10 times higher.[3]Dummer R, Vermeer MH, Scarisbrick JJ, et al. Cutaneous T cell lymphoma. Nat Rev Dis Primers. 2021 Aug 26;7(1):61.
http://www.ncbi.nlm.nih.gov/pubmed/34446710?tool=bestpractice.com
Approximately 70% of primary cutaneous lymphomas are of T-cell origin, of which the majority are mycosis fungoides (MF) and its variant Sézary syndrome (SS).[1]Olsen EA, Whittaker S, Kim YH, et al. Clinical end points and response criteria in mycosis fungoides and Sézary syndrome: a consensus statement of the International Society for Cutaneous Lymphomas, the United States Cutaneous Lymphoma Consortium, and the Cutaneous Lymphoma Task Force of the European Organisation for Research and Treatment of Cancer. J Clin Oncol. 2011 Jun 20;29(18):2598-607.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3422534
http://www.ncbi.nlm.nih.gov/pubmed/21576639?tool=bestpractice.com
[4]Bradford PT, Devesa SS, Anderson WF, et al. Cutaneous lymphoma incidence patterns in the United States: a population-based study of 3884 cases. Blood. 2009 May 21;113(21):5064-73.
https://www.doi.org/10.1182/blood-2008-10-184168
http://www.ncbi.nlm.nih.gov/pubmed/19279331?tool=bestpractice.com
[5]Gilson D, Whittaker SJ, Child FJ, et al. Joint British Association of Dermatologists and UK Cutaneous Lymphoma Group guidelines for the management of primary cutaneous T-cell lymphomas. Br J Dermatol. 2019 Mar;180(3):496-526.
https://onlinelibrary.wiley.com/doi/10.1111/bjd.17240
http://www.ncbi.nlm.nih.gov/pubmed/30561020?tool=bestpractice.com
Evidence suggests that the incidence of MF and SS does not vary between regions. MF and SS are somewhat more common in males, with a male to female ratio of 1.7:1.[3]Dummer R, Vermeer MH, Scarisbrick JJ, et al. Cutaneous T cell lymphoma. Nat Rev Dis Primers. 2021 Aug 26;7(1):61.
http://www.ncbi.nlm.nih.gov/pubmed/34446710?tool=bestpractice.com
Age of presentation is typically 50-60 years for early stage disease, and 60-70 years for advanced disease.[3]Dummer R, Vermeer MH, Scarisbrick JJ, et al. Cutaneous T cell lymphoma. Nat Rev Dis Primers. 2021 Aug 26;7(1):61.
http://www.ncbi.nlm.nih.gov/pubmed/34446710?tool=bestpractice.com
Approximately 20% of patients are diagnosed with CTCL in the 25-49 age group, with the same male preponderance.[4]Bradford PT, Devesa SS, Anderson WF, et al. Cutaneous lymphoma incidence patterns in the United States: a population-based study of 3884 cases. Blood. 2009 May 21;113(21):5064-73.
https://www.doi.org/10.1182/blood-2008-10-184168
http://www.ncbi.nlm.nih.gov/pubmed/19279331?tool=bestpractice.com
[6]Saunes M, Nilsen TI, Johannesen TB. Incidence of primary cutaneous T-cell lymphoma in Norway. Br J Dermatol. 2009 Feb;160(2):376-9.
http://www.ncbi.nlm.nih.gov/pubmed/18808419?tool=bestpractice.com
People of non-white heritage may present with CTCL earlier, and there may be higher incidence rates of MF in Asian populations.[3]Dummer R, Vermeer MH, Scarisbrick JJ, et al. Cutaneous T cell lymphoma. Nat Rev Dis Primers. 2021 Aug 26;7(1):61.
http://www.ncbi.nlm.nih.gov/pubmed/34446710?tool=bestpractice.com
Some data have demonstrated that the incidence of CTCL is higher among African-Americans when compared with white Americans and other racial groups; MF is twice as common among black Americans compared with white Americans; and that SS appears to be more common among white Americans.[7]Hinds GA, Heald P. Cutaneous T-cell lymphoma in skin of color. J Am Acad Dermatol. 2009 Mar;60(3):359-75; quiz 376-8.
http://www.ncbi.nlm.nih.gov/pubmed/19231637?tool=bestpractice.com
[8]Wu XC, Andrews P, Chen VW, et al. Incidence of extranodal non-Hodgkin lymphomas among whites, blacks, and Asians/Pacific Islanders in the United States: anatomic site and histology differences. Cancer Epidemiol. 2009 Nov;33(5):337-46.
http://www.ncbi.nlm.nih.gov/pubmed/19853554?tool=bestpractice.com
[9]Weinstock MA, Horm JW. Mycosis fungoides in the United States. Increasing incidence and descriptive epidemiology. JAMA. 1988 Jul 1;260(1):42-6.
http://www.ncbi.nlm.nih.gov/pubmed/3379722?tool=bestpractice.com
[10]Crandon S, Yancey MA. Sezary syndrome: a case study of cutaneous T-cell lymphoma. Clin J Oncol Nurs. 2009 Apr;13(2):157-9.
http://www.ncbi.nlm.nih.gov/pubmed/19349262?tool=bestpractice.com
The incidence of early-stage MF has been reported as 0.26 per 100,000 in the UK, and 0.38 per 100,000 in the US based on data from SEER, the prevalence was 4.8 per 100,000, and 6.6 per 100,000, respectively.[11]Maguire A, Puelles J, Raboisson P, et al. Early-stage mycosis fungoides: epidemiology and prognosis. Acta Derm Venereol. 2020 Jan 7;100(1):adv00013.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9128921
http://www.ncbi.nlm.nih.gov/pubmed/31663598?tool=bestpractice.com