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T-cell Lymphoma Epidemiology: the Known and Unknown

  • T-Cell and Other Lymphoproliferative Malignancies (P Porcu, Section Editor)
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Abstract

T-cell lymphoma, a collection of subtypes of Non-Hodgkin lymphoma, is a rare malignancy. The low prevalence of this disease has made it challenging to identify subtype-specific risk factors. Potential risk factors could enable us to identify high-risk patients and predict patient outcomes. Here, we report on the current epidemiologic and prognostic factors data associated with the individual subtypes both of peripheral T-cell lymphoma (PTCL) and cutaneous T-cell lymphoma (CTCL) found in large cohort and case studies. Additionally, with recent findings, as well as updates in the new World Health Organization (WHO) classification of lymphoid neoplasms, we consider what this could do to change our approach to this group of diseases.

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References

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  1. Surveillance, Epidemiology, and End Results (SEER) Program. National Cancer Instutite, DCCPS, Cancer Statistics Branch 2013; Available from: www.seer.cancer.gov [updated based on the November 2013 submission].

  2. Whang-Peng J, Bunn Jr PA, Knutsen T, Matthews MJ, Schechter G, et al. Clinical implications of cytogenetic studies in cutaneous T-cell lymphoma (CTCL). Cancer. 1982;50(8):1539–53.

    Article  CAS  PubMed  Google Scholar 

  3. Pinkus GS, Said JW, Hargreaves H. Malignant lymphoma, T-cell type A distinct morphologic variant with large multilobated nuclei, with a report of four cases. Am J Clin Pathol. 1979;72(4):540–50.

    Article  CAS  PubMed  Google Scholar 

  4. Wang SS, Vose JM. Epidemiology and Prognosis of T-Cell Lymphoma. In: Foss, F, editor. T-cell Lymphomas. Humana Press; 2013. p. 25–39, Totowa, NJ: Humana Press

  5. The NHL Classification Project. A clinical evaluation of the International Lymphoma Study Group classification of non-Hodgkin’s lymphoma. The Non-Hodgkin’s Lymphoma Classification Project. Blood. 1997;89(11):3909–18.

    Google Scholar 

  6. Lymphoma.org- http://www.lymphoma.org/site/pp.asp?c=bkLTKaOQLmK8E&b=6300161.

  7. Geskin Larisa J. Chapter 105. Cutaneous T-cell lymphoma (mycosis fungoides and Sézary syndrome). In: Lichtman MA, Kipps TJ, Seligsohn U, editors. Williams Hematology. 8th ed. Available from: AccessMedicine. Accessed 1 Aug 2016.

  8. Swerdlow SH et al. The 2016 revision of the World Health Organization classification of lymphoid neoplasms. Blood. 2016;127(20):2375–90. Highlighted the changes made in the updated 2016 World Health Organization (WHO) classification of lymphoid neoplasm, in terms of categorize of subtypes and clinical expectations.

    Article  PubMed  Google Scholar 

  9. Vose J et al. International peripheral T-cell and natural killer/T-cell lymphoma study: pathology findings and clinical outcomes. J Clin Oncol. 2008;26(25):4124–30.

    Article  PubMed  Google Scholar 

  10. Morton LM et al. Rationale and design of the International Lymphoma Epidemiology Consortium (InterLymph) Non-Hodgkin Lymphoma Subtypes Project. J Natl Cancer Inst Monogr. 2014;2014(48):1–14.

    Article  PubMed  PubMed Central  Google Scholar 

  11. Savage KJ, Chhanabhai M, Gascoyne RD, Connors JM. Characterization of peripheral T-cell lymphomas in a single North American institution by the WHO classification. Ann Oncol. 2004;15:1467–75. doi:10.1093/annonc/mdh392.

    Article  CAS  PubMed  Google Scholar 

  12. Abramson JS et al. Peripheral T-cell lymphomas in a large US multicenter cohort: prognostication in the modern era including impact of frontline therapy. Ann Oncol. 2014;25(11):2211–7.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  13. Smith A et al. Lymphoma incidence, survival and prevalence 2004-2014: sub-type analyses from the UK’s Haematological Malignancy Research Network. Br J Cancer. 2015;112(9):1575–84.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  14. Adams SV et al. Racial patterns of peripheral T-cell lymphoma incidence and survival in the United States. J Clin Oncol. 2016;34(9):963–71. Using SEER data from 2000-2012, this study examine the latest trends for each subtypes, in terms of age of diagnosis, survival rates, incidences rates, and case-case odd ratios by each.

    Article  PubMed  PubMed Central  Google Scholar 

  15. Wang SS et al. Medical history, lifestyle, family history, and occupational risk factors for peripheral T-cell lymphomas: the InterLymph Non-Hodgkin Lymphoma Subtypes Project. J Natl Cancer Inst Monogr. 2014;2014(48):66–75.

    Article  PubMed  PubMed Central  Google Scholar 

  16. Koff JL et al. To each its own: linking the biology and epidemiology of NHL subtypes. Curr Hematol Malig Rep. 2015;10(3):244–55.

    Article  PubMed  Google Scholar 

  17. Park S, Ko YH. Peripheral T cell lymphoma in Asia. Int J Hematol. 2014;99(3):227–39.

    Article  CAS  PubMed  Google Scholar 

  18. Ai WZ et al. Racial patterns of extranodal natural killer/T-cell lymphoma, nasal type, in California: a population-based study. Br J Haematol. 2012;156(5):626–32.

    Article  CAS  PubMed  Google Scholar 

  19. Perry AM et al. Non-Hodgkin lymphoma in the Far East: review of 730 cases from the International Non-Hodgkin Lymphoma Classification Project. Ann Hematol. 2016;95(2):245–51.

    Article  PubMed  Google Scholar 

  20. Au WY et al. Clinicopathologic features and treatment outcome of mature T-cell and natural killer-cell lymphomas diagnosed according to the World Health Organization classification scheme: a single center experience of 10 years. Ann Oncol. 2005;16(2):206–14.

    Article  PubMed  Google Scholar 

  21. Lim ST et al. Comparative analysis of extra-nodal NK/T-cell lymphoma and peripheral T-cell lymphoma: significant differences in clinical characteristics and prognosis. Eur J Haematol. 2008;80(1):55–60.

    PubMed  Google Scholar 

  22. Tang T et al. Peripheral T-cell lymphoma: review and updates of current management strategies. Adv Hematol. 2010;2010:624040.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  23. Cahoon EK et al. Relationship between ambient ultraviolet radiation and non-Hodgkin lymphoma subtypes: a U.S. population-based study of racial and ethnic groups. Int J Cancer. 2015;136(5):E432–41.

    Article  CAS  PubMed  Google Scholar 

  24. Beltran BE et al. The neutrophil-to-lymphocyte ratio is an independent prognostic factor in patients with peripheral T-cell lymphoma, unspecified. Leuk Lymphoma. 2016;57(1):58–62.

    Article  PubMed  Google Scholar 

  25. de Leval L et al. Angioimmunoblastic T-cell lymphoma is the most common T-cell lymphoma in two distinct French information data sets. Haematologica. 2015;100(9):e361–4.

  26. Khan G et al. Epstein-Barr virus in angioimmunoblastic T-cell lymphomas. Histopathology. 1993;22(2):145–9.

    Article  CAS  PubMed  Google Scholar 

  27. Beer T, Dorion P. Angioimmunoblastic T-cell lymphoma presenting with an acute serologic Epstein-Barr virus profile. Hematol Rep. 2015;7(2):5893.

    Article  PubMed  PubMed Central  Google Scholar 

  28. Fallah M et al. Autoimmune diseases associated with non-Hodgkin lymphoma: a nationwide cohort study. Ann Oncol. 2014;25(10):2025–30.

    Article  CAS  PubMed  Google Scholar 

  29. Quintanilla-Martinez L, Jansen PM, Kinney MC, Swerdlow SH, Willemze R. Non-mycosis fungoides cutaneous T-cell lymphomas: report of the 2011 Society for Hematopathology/European Association for Haematopathology workshop. Am J Clin Pathol. 2013;139(4):491–514.

    Article  CAS  PubMed  Google Scholar 

  30. Willemze R, Jaffe ES, Burg G, Cerroni L, Berti E, et al. WHO-EORTC classification for cutaneous lymphomas. Blood. 2005;105(10):3768–85.

    Article  CAS  PubMed  Google Scholar 

  31. Benner MF, Jansen PM, Vermeer MH, Willemze R. Prognostic factors in transformed mycosis fungoides: a retrospective analysis of 100 cases. Blood. 2012;119(7):1643–9.

    Article  CAS  PubMed  Google Scholar 

  32. Kempf W, Kazakov DV, Kerl K. Cutaneous lymphomas: an update. Part 1: T-cell and natural killer/t-cell lymphomas and related conditions. Am J Dermatopathol. 2014;36(2):105–23.

    Article  PubMed  Google Scholar 

  33. Kadin ME, Pinkus JL, Pinkus GS, Duran IH, Fuller CE, et al. Primary cutaneous ALCL with phosphorylated/activated cytoplasmic ALK and novel phenotype: EMA/MUC1+, cutaneous lymphocyte antigen negative. Am J Surg Pathol. 2008;32(9):1421–6.

    Article  PubMed  Google Scholar 

  34. de Baaij LR et al. A new and validated clinical prognostic model (EPI) for enteropathy-associated T-cell lymphoma. Clin Cancer Res. 2015;21(13):3013–9. A respective study showed that the current prognostic model, IPI, to identify high risk patients with enteropathy-associated T-cell lymphoma can be improved by including B-symptoms.

    Article  PubMed  CAS  Google Scholar 

  35. Burg G et al. WHO/EORTC classification of cutaneous lymphomas 2005: histological and molecular aspects. J Cutan Pathol. 2005;32(10):647–74.

    Article  PubMed  Google Scholar 

  36. Paulli M, Berti E. Cutaneous T-cell lymphomas (including rare subtypes). Current concepts II. Haematologica. 2004;89(11):1372–88.

    PubMed  Google Scholar 

  37. Sidiropoulos KG, Martinez-Escala ME, Yelamos O, Guitart J, Sidiropoulos M. Primary cutaneous T-cell lymphomas: a review. J Clin Pathol. 2015;68(12):1003–10.

    Article  CAS  PubMed  Google Scholar 

  38. Yoshida N, Chihara D. Incidence of adult T-cell leukemia/lymphoma in nonendemic areas. Curr Treat Options in Oncol. 2015;16(7):1–8.

    Google Scholar 

  39. Proietti FA et al. Global epidemiology of HTLV-I infection and associated diseases. Oncogene. 2005;24(39):6058–68.

    Article  CAS  PubMed  Google Scholar 

  40. Hamada T, Iwatsuki K. Cutaneous lymphoma in Japan: a nationwide study of 1733 patients. J Dermatol. 2014;41(1):3–10.

    Article  CAS  PubMed  Google Scholar 

  41. Kawano N et al. Clinical features and treatment outcomes of 81 patients with aggressive type adult T-cell leukemia-lymphoma at a single institution over a 7-year period (2006-2012). Intern Med. 2015;54(12):1489–98.

    Article  PubMed  Google Scholar 

  42. Ohshima K. Molecular pathology of adult T-cell leukemia/lymphoma. Oncology. 2015;89 Suppl 1:7–15.

    Article  PubMed  Google Scholar 

  43. Iwanaga M, Watanabe T, Yamaguchi K. Adult T-cell leukemia: a review of epidemiological evidence. Front Microbiol. 2012;3:322.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  44. Karaarslan S, Hekimgil M, Soydan S, Ertan Y, Doganavargil B. Evaluation of the role of Epstein-Barr virus in cases of nodal or extranodal T- and NK-cell lymphoma using eber in situ hybridization. Pol J Pathol. 2015;66(2):161–9. doi:10.5114/pjp.2015.53013.

    Article  PubMed  Google Scholar 

  45. Cai Q et al. Fasting blood glucose is a novel prognostic indicator for extranodal natural killer/T-cell lymphoma, nasal type. Br J Cancer. 2013;108(2):380–6.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  46. Foppoli M, Ferreri AJ. Gamma-delta t-cell lymphomas. Eur J Haematol. 2015;94(3):206–18.

    Article  CAS  PubMed  Google Scholar 

  47. Samimi S. Update on epidemiology of cutaneous T-cell lymphoma. Curr Dermatol Rep. 2013;2:35–41.

    Article  Google Scholar 

  48. 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(21):5064–73.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  49. Korgavkar K, Xiong M, Weinstock M. Changing incidence trends of cutaneous T-cell lymphoma. JAMA Dermatol. 2013;149(11):1295–9.

    Article  PubMed  Google Scholar 

  50. Imam MH, Shenoy PJ, Flowers CR, Phillips A, Lechowicz MJ. Incidence and survival patterns of cutaneous T-cell lymphomas in the United States. Leuk Lymphoma. 2013;54(4):752–9.

    Article  PubMed  Google Scholar 

  51. Criscione VD, Weinstock MA. Incidence of cutaneous T-cell lymphoma in the United States, 1973-2002. Arch Dermatol. 2007;143(7):854–9.

    Article  PubMed  Google Scholar 

  52. Litvinov IV, Tetzlaff MT, Rahme E, Habel Y, Risser DR, et al. Identification of geographic clustering and regions spared by cutaneous T-cell lymphoma in Texas using 2 distinct cancer registries. Cancer. 2015;121(12):1993–2003.

    Article  PubMed  PubMed Central  Google Scholar 

  53. Dupin M, Darie H, Jumbou O, Veran Y, Gros P, et al. Conjugal mycosis fungoides. Ann Dermatol Venereol. 1995;122(9):595–8.

    CAS  PubMed  Google Scholar 

  54. Schmidt AN, Robbins JB, Greer JP, Zic JA. Conjugal transformed mycosis fungoides: the unknown role of viral infection and environmental exposures in the development of cutaneous T-cell lymphoma. J Am Acad Dermatol. 2006;54(5 Suppl):S202–5.

    Article  PubMed  Google Scholar 

  55. Lozano A, Duvic M. Cutaneous T-cell lymphoma in non-blood-related family members: report of an additional case. J Am Acad Dermatol. 2007;56(3):521.

    Article  PubMed  Google Scholar 

  56. Hodak E, Friedman E. Familial mycosis fungoides: model of genetic susceptibility. Clin Lymphoma Myeloma Leuk. 2010;10 Suppl 2:S67–9.

    Article  PubMed  Google Scholar 

  57. Weder P, Anliker M, Itin P, Bargetzi M. Familial cutaneous mycosis fungoides: successful treatment with a combination of gemcitabine and alemtuzumab. Dermatology. 2004;208(3):281–3.

    Article  CAS  PubMed  Google Scholar 

  58. Pomerantz RG, Campbell LS, Jukic DM, Geskin LJ. Posttransplant cutaneous T-cell lymphoma: case reports and review of the association of calcineurin inhibitor use with posttransplant lymphoproliferative disease risk. Arch Dermatol. 2010;146(5):513–6.

    Article  PubMed  Google Scholar 

  59. Wilkins K, Turner R, Dolev JC, LeBoit PE, Berger TG, et al. Cutaneous malignancy and human immunodeficiency virus disease. J Am Acad Dermatol. 2006;54(2):189–206. quiz 207-10.

    Article  PubMed  Google Scholar 

  60. Biggar RJ, Engels EA, Frisch M, Goedert JJ. Risk of T-cell lymphomas in persons with AIDS. J Acquir Immune Defic Syndr. 2001;26(4):371–6.

    Article  CAS  PubMed  Google Scholar 

  61. Jackow CM, McHam JB, Friss A, Alvear J, Reveille JR, et al. HLA-DR5 and DQB1*03 class II alleles are associated with cutaneous T-cell lymphoma. J Invest Dermatol. 1996;107(3):373–6.

    Article  CAS  PubMed  Google Scholar 

  62. Safai B, Myskowski PL, Dupont B, Pollack MS. Association of HLA-DR5 with mycosis fungoides. J Invest Dermatol. 1983;80(5):395–7.

    Article  CAS  PubMed  Google Scholar 

  63. Mirvish JJ, Pomerantz RG, Falo Jr LD, Geskin LJ. Role of infectious agents in cutaneous T-cell lymphoma: facts and controversies. Clin Dermatol. 2013;31(4):423–31.

    Article  PubMed  Google Scholar 

  64. Wood GS, Salvekar A, Schaffer J, Crooks CF, Henghold W, et al. Evidence against a role for human T-cell lymphotrophic virus type I (HTLV-I) in the pathogenesis of American cutaneous T-cell lymphoma. J Invest Dermatol. 1996;107(3):301–7.

    Article  CAS  PubMed  Google Scholar 

  65. Bazarbachi A, Saal F, Laroche L, Flageul B, Périès J, et al. HTLV-1 provirus and mycosis fungoides. Science. 1993;259(5100):1470–1.

    Article  CAS  PubMed  Google Scholar 

  66. Courgnaud V, Duthanh A, Guillot B, Sitbon M, Dereure O. Absence of HTLV-related sequences in skin lesions and peripheral blood of cutaneous T-cell lymphomas. J Invest Dermatol. 2009;129(10):2520–2.

    Article  CAS  PubMed  Google Scholar 

  67. Jawed SI, Myskowski PL, Horwitz S, Moskowitz A, Querfeld C. Primary cutaneous T-cell lymphoma (mycosis fungoides and Sézary syndrome): part I. Diagnosis: clinical and histopathologic features and new molecular and biologic markers. J Am Acad Dermatol. 2014;70(2):205.e1-16. quiz 221-2.

    Article  Google Scholar 

  68. Zucker-Franklin D, Hooper WC, Evatt BL. Human lymphotropic retroviruses associated with mycosis fungoides: evidence that human T-cell lymphotropic virus type II (HTLV-II) as well as HTLV-I may play a role in the disease. Blood. 1992;80(6):1537–45.

    CAS  PubMed  Google Scholar 

  69. Pancake BA, Zucker-Franklin D, Coutavas EE. The cutaneous T cell lymphoma, mycosis fungoides, is a human T cell lymphotropic virus-associated disease. A study of 50 patients. J Clin Invest. 1995;95(2):547–54.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  70. Detmar M, Pauli G, Anagnostopoulos I, Wunderlich U, Herbst H, et al. A case of classical mycosis fungoides associated with human T-cell lymphotropic virus type I. Br J Dermatol. 1991;124(2):198–202.

    Article  CAS  PubMed  Google Scholar 

  71. Burg G. Systemic involvement in mycosis fungoides. Clin Dermatol. 2015;33(5):563–71.

    Article  PubMed  Google Scholar 

  72. Campbell JJ, Clark RA, Watanabe R, Kupper TS. Sezary syndrome and mycosis fungoides arise from distinct T-cell subsets: a biologic rationale for their distinct clinical behaviors. Blood. 2010;116(5):767–71.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  73. Wong HK, Mishra A, Hake T, Porcu P. Evolving insights in the pathogenesis and therapy of cutaneous T-cell lymphoma (mycosis fungoides and Sezary syndrome). Br J Haematol. 2011;155(2):150–66.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  74. Scarisbrick JJ, Kim YH, Whittaker SJ, Wood GS, Vermeer MH, et al. Prognostic factors, prognostic indices and staging in mycosis fungoides and Sézary syndrome: where are we now? Br J Dermatol. 2014;170(6):1226–36.

    Article  CAS  PubMed  Google Scholar 

  75. Sun G, Berthelot C, Li Y, Glass 2nd DA, George D, et al. Poor prognosis in non-Caucasian patients with early-onset mycosis fungoides. J Am Acad Dermatol. 2009;60(2):231–5.

    Article  PubMed  Google Scholar 

  76. Wilson LD, Hinds GA, Yu JB. Age, race, sex, stage, and incidence of cutaneous lymphoma. Clin Lymphoma Myeloma Leuk. 2012;12(5):291–6.

    Article  PubMed  PubMed Central  Google Scholar 

  77. Alsaleh QA, Nanda A, Al-Ajmi H, Al-Sabah H, Elkashlan M, et al. Clinicoepidemiological features of mycosis fungoides in Kuwait, 1991-2006. Int J Dermatol. 2010;49(12):1393–8.

    Article  PubMed  Google Scholar 

  78. Tan ES, Tang MB, Tan SH. Retrospective 5-year review of 131 patients with mycosis fungoides and Sézary syndrome seen at the National Skin Centre, Singapore. Australas J Dermatol. 2006;47(4):248–52.

    Article  PubMed  Google Scholar 

  79. Morales Suárez-Varela MM, Olsen J, Kaerlev L, Guénel P, Arveux P, et al. Are alcohol intake and smoking associated with mycosis fungoides? A European multicentre case-control study. Eur J Cancer. 2001;37(3):392–7.

    Article  Google Scholar 

  80. Jang MS, Jang JG, Han SH, Park JB, Kang DY, et al. Clinicopathological features of mycosis fungoides in patients exposed to Agent Orange during the Vietnam War. J Dermatol. 2013;40(8):606–12.

    Article  PubMed  Google Scholar 

  81. Bonin S, Tothova SM, Barbazza R, Brunetti D, Stanta G, Trevisan G. Evidence of multiple infectious agents in mycosis fungoides lesions. Exp Mol Pathol. 2010;89(1):46–50. doi:10.1016/j.yexmp.2010.05.001.

    Article  CAS  PubMed  Google Scholar 

  82. Aschebrook-Kilfoy B, Cocco P, La Vecchia C, Chang ET, Vajdic CM, et al. Medical history, lifestyle, family history, and occupational risk factors for mycosis fungoides and Sézary syndrome: the InterLymph Non-Hodgkin Lymphoma Subtypes Project. J Natl Cancer Inst Monogr. 2014;2014(48):98–105.

    Article  PubMed  PubMed Central  Google Scholar 

  83. Fava P, Bergallo M, Astrua C, Brizio M, Galliano I, et al. Human endogenous retrovirus expression in primary cutaneous T-cell lymphomas. Dermatology. 2016;232(1):38–43.

    Article  CAS  PubMed  Google Scholar 

  84. Jahan-Tigh RR, Huen AO, Lee GL, Pozadzides JV, Liu P, et al. Hydrochlorothiazide and cutaneous T cell lymphoma: prospective analysis and case series. Cancer. 2013;119(4):825–31.

    Article  CAS  PubMed  Google Scholar 

  85. Van Santen S, Roach RE, van Doorn R, Horváth B, Bruijn MS, et al. Clinical staging and prognostic factors in folliculotropic mycosis fungoides. JAMA Dermatol. 2016;152(9):992–1000.

  86. Van Doorn R, Van Haselen CW, van Voorst Vader PC, Geerts ML, Heule F, et al. Mycosis fungoides: disease evolution and prognosis of 309 Dutch patients. Arch Dermatol. 2000;136(4):504–10.

    PubMed  Google Scholar 

  87. Benton EC, Crichton S, Talpur R, Agar NS, Fields PA, et al. A cutaneous lymphoma international prognostic index (CLIPi) for mycosis fungoides and Sezary syndrome. Eur J Cancer. 2013;49(13):2859–68.

    Article  CAS  PubMed  Google Scholar 

  88. Agar NS, Wedgeworth E, Crichton S, Mitchell TJ, Cox M, et al. Survival outcomes and prognostic factors in mycosis fungoides/Sézary syndrome: validation of the revised International Society for Cutaneous Lymphomas/European Organisation for Research and Treatment of Cancer staging proposal. J Clin Oncol. 2010;28(31):4730–9.

    Article  PubMed  Google Scholar 

  89. Scarisbrick JJ et al. Cutaneous lymphoma international consortium study of outcome in advanced stages of mycosis fungoides and sezary syndrome: effect of specific prognostic markers on survival and development of a prognostic model. J Clin Oncol. 2015;33(32):3766–73.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  90. Li JY et al. A case-control study of clinicopathologic features, prognosis, and therapeutic responses in patients with granulomatous mycosis fungoides. J Am Acad Dermatol. 2013;69(3):366–74.

    Article  PubMed  Google Scholar 

  91. Kempf W et al. Granulomatous mycosis fungoides and granulomatous slack skin: a multicenter study of the Cutaneous Lymphoma Histopathology Task Force Group of the European Organization For Research and Treatment of Cancer (EORTC). Arch Dermatol. 2008;144(12):1609–17.

    Article  PubMed  Google Scholar 

  92. Abbott RA, Sahni D, Robson A, Agar N, Whittaker S, et al. Poikilodermatous mycosis fungoides: a study of its clinicopathological, immunophenotypic, and prognostic features. J Am Acad Dermatol. 2011;65(2):313–9.

    Article  PubMed  Google Scholar 

  93. Zackheim HS, McCalmont TH, Deanovic FW, Odom RB. Mycosis fungoides with onset before 20 years of age. J Am Acad Dermatol. 1997;36(4):557–62.

    Article  CAS  PubMed  Google Scholar 

  94. Assaf C, Gellrich S, Steinhoff M, Nashan D, Weisse F, et al. Cutaneous lymphomas in Germany: an analysis of the Central Cutaneous Lymphoma Registry of the German Society of Dermatology (DDG). J Dtsch Dermatol Ges. 2007;5(8):662–8.

    Article  PubMed  Google Scholar 

  95. Nanda A, AlSaleh QA, Al-Ajmi H, Al-Sabah H, Elkashlan M, et al. Mycosis fungoides in Arab children and adolescents: a report of 36 patients from Kuwait. Pediatr Dermatol. 2010;27(6):607–13.

    Article  PubMed  Google Scholar 

  96. Hodak E, Amitay-Laish I, Feinmesser M, Davidovici B, David M, et al. Juvenile mycosis fungoides: cutaneous T-cell lymphoma with frequent follicular involvement. J Am Acad Dermatol. 2014;70(6):993–1001.

    Article  PubMed  Google Scholar 

  97. Laharanne E, Oumouhou N, Bonnet F, Carlotti M, Gentil C, et al. Genome-wide analysis of cutaneous T-cell lymphomas identifies three clinically relevant classes. J Invest Dermatol. 2010;130(6):1707–18.

    Article  CAS  PubMed  Google Scholar 

  98. Abdul Samad K, Prasanna MK, Akhar AP. Sezary syndrome—without erythroderma. Indian J Dermatol Venereol Leprol. 2002;68(4):225–6.

    CAS  PubMed  Google Scholar 

  99. DeSimone JA, Sodha P, Ignatova D, Dummer R, Cozzio A, et al. Recent advances in primary cutaneous T-cell lymphoma. Curr Opin Oncol. 2015;27(2):128–33.

    Article  CAS  PubMed  Google Scholar 

  100. Desai M et al. Clinical characteristics, prognostic factors, and survival of 393 patients with mycosis fungoides and Sezary syndrome in the southeastern United States: a single-institution cohort. J Am Acad Dermatol. 2015;72(2):276–85.

    Article  PubMed  Google Scholar 

  101. Willemze R et al. Primary cutaneous lymphomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2013;24 Suppl 6:vi149–54.

    Article  PubMed  Google Scholar 

  102. de Souza A et al. In search of prognostic indicators for lymphomatoid papulosis: a retrospective study of 123 patients. J Am Acad Dermatol. 2012;66(6):928–37.

    Article  PubMed  Google Scholar 

  103. Wang HH, Lach L, Kadin ME. Epidemiology of lymphomatoid papulosis. Cancer. 1992;70(12):2951–7.

    Article  CAS  PubMed  Google Scholar 

  104. Talpur R, Singh L, Daulat S, Liu P, Seyfer S, et al. Long-term outcomes of 1,263 patients with mycosis fungoides and Sézary syndrome from 1982 to 2009. Clin Cancer Res. 2012;18(18):5051–60.

    Article  CAS  PubMed  Google Scholar 

  105. Wieser I, Oh CW, Talpur R, Duvic M. Lymphomatoid papulosis: treatment response and associated lymphomas in a study of 180 patients. J Am Acad Dermatol. 2016;74(1):59–67.

    Article  PubMed  Google Scholar 

  106. Kempf W, Kazakov DV, Belousova IE, Mitteldorf C, Kerl K. Paediatric cutaneous lymphomas: a review and comparison with adult counterparts. J Eur Acad Dermatol Venereol. 2015;29(9):1696–709.

    Article  CAS  PubMed  Google Scholar 

  107. Talpur R et al. Retrospective analysis of prognostic factors in 187 cases of transformed mycosis fungoides. Clin Lymphoma Myeloma Leuk. 2016;16(1):49–56.

    Article  PubMed  Google Scholar 

  108. Iqbal K, Bott J, Robson A, Post R, Morris S, et al. Nonerythrodermic Sézary syndrome. Clin Exp Dermatol. 2011;36(2):207–8.

    Article  CAS  PubMed  Google Scholar 

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Correspondence to Mary Jo Lechowicz.

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Anh Phan and Rachel Veldman each declare no potential conflicts of interest.

Mary Jo Lechowicz is on the advisory boards of Spectrum Pharmaceuticals and Soligenix, Inc.

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This article is part of the Topical Collection on T-Cell and Other Lymphoproliferative Malignancies

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Phan, A., Veldman, R. & Lechowicz, M.J. T-cell Lymphoma Epidemiology: the Known and Unknown. Curr Hematol Malig Rep 11, 492–503 (2016). https://doi.org/10.1007/s11899-016-0353-y

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