Epidemiology and Prognosis of T-Cell Lymphoma

  • Sophia S. Wang
  • Julie M. Vose
Part of the Contemporary Hematology book series (CH)


T/NK-cell lymphoid neoplasms account for approximately 6 % of all lymphoid neoplasms. While the incidence rates of many B-cell lymphomas have begun to decline in the United States (US) in recent years, incidence rates for T-cell lymphomas have continued to rise. The causes of the majority of T-cell lymphomas remain unexplained. T-cell lymphomas comprise multiple subtypes and their distinct descriptive epidemiology—e.g., striking differences between incidences of T-cell lymphoma subtypes by age, over time and by race/ethnicity—likely reflect their distinct etiologies (e.g., celiac disease and extranodal T-cell lymphomas; human T-cell lymphotropic virus I (HTLV-1) and adult T-cell leukemia/lymphoma). Differences in T-cell lymphoma subtypes further extend to their clinical characteristics and care; they each have differential treatment, survival and prognosis. The rarity of T-cell lymphomas has historically posed challenges for furthering our understanding of these tumors, particularly as we now understand that each T-cell lymphoma subtype requires investigation independently. However, we expect important clues to emerge as on-going large international consortium efforts aim to accrue sufficient sample sizes of T-cell lymphomas and its subtypes for both etiological and prognostic studies.


Celiac Disease Anaplastic Lymphoma Kinase Anaplastic Large Cell Lymphoma Mycosis Fungoides Lymphoid Neoplasm 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.



We thank J. Daniel Carreon for guidance on descriptive statistics and graphical presentation.


  1. 1.
    Au WY, Lo J. HTLV-1-related lymphoma in Hong Kong Chinese. Am J Hematol. 2005;78:80–1.PubMedCrossRefGoogle Scholar
  2. 2.
    Naresh KN, Srinivas V, Soman CS. Distribution of various subtypes of non-Hodgkin’s lymphoma in India: a study of 2773 lymphomas using R.E.A.L. and WHO Classifications. Ann Oncol. 2000;11:63–7.PubMedCrossRefGoogle Scholar
  3. 3.
    Ng CS, Chan J, Lo S, Poon Y. Immunophenotypic analysis of non-Hodgkin’s lymphomas in Chinese. A study of 75 cases in Hong Kong. Pathology. 1986;18:419–25.PubMedCrossRefGoogle Scholar
  4. 4.
    Kadin ME, Berard CW, Nanba K, Wakasa H. Lymphoproliferative diseases in Japan and Western countries: Proceedings of the United States—Japan Seminar, September 6 and 7, 1982, in Seattle, Washington. Hum Pathol. 1983;14:745–72.PubMedCrossRefGoogle Scholar
  5. 5.
    Carreon JD, Morton LM, Devesa SS, Clarke CA, Gomez SL, Glaser SL, et al. Incidence of lymphoid neoplasms by subtype among six Asian ethnic groups in the United States, 1996–2004. Cancer Causes Control. 2008;19:1171–81.PubMedCrossRefGoogle Scholar
  6. 6.
    Goldin LR, Landgren O, McMaster ML, Gridley G, Hemminki K, Li X, et al. Familial aggregation and heterogeneity of non-Hodgkin lymphoma in population-based samples. Cancer Epidemiol Biomarkers Prev. 2005;14:2402–6.PubMedCrossRefGoogle Scholar
  7. 7.
    Goldgar DE, Easton DF, Cannon-Albright LA, Skolnick MH. Systematic population-based assessment of cancer risk in first-degree relatives of cancer probands. J Natl Cancer Inst. 1994;86:1600–8.PubMedCrossRefGoogle Scholar
  8. 8.
    Wang SS, Slager SL, Brennan P, Holly EA, De SS, Bernstein L, et al. Family history of hematopoietic malignancies and risk of non-Hodgkin lymphoma (NHL): a pooled analysis of 10 211 cases and 11 905 controls from the International Lymphoma Epidemiology Consortium (InterLymph). Blood. 2007;109:3479–88.PubMedCrossRefGoogle Scholar
  9. 9.
    Friedman DL, Kadan-Lottick NS, Whitton J, Mertens AC, Yasui Y, Liu Y, et al. Increased risk of cancer among siblings of long-term childhood cancer survivors: a report from the childhood cancer survivor study. Cancer Epidemiol Biomarkers Prev. 2005;14:1922–7.PubMedCrossRefGoogle Scholar
  10. 10.
    Huang KP, Weinstock MA, Clarke CA, McMillan A, Hoppe RT, Kim YH. Second lymphomas and other malignant neoplasms in patients with mycosis fungoides and Sezary syndrome: evidence from population-based and clinical cohorts. Arch Dermatol. 2007;143:45–50.PubMedCrossRefGoogle Scholar
  11. 11.
    Ehrenfeld M, Abu-Shakra M, Buskila D, Shoenfeld Y. The dual association between lymphoma and autoimmunity. Blood Cells Mol Dis. 2001;27:750–6.PubMedCrossRefGoogle Scholar
  12. 12.
    Corrao G, Corazza GR, Bagnardi V, Brusco G, Ciacci C, Cottone M, et al. Mortality in patients with coeliac disease and their relatives: a cohort study. Lancet. 2001;358:356–61.PubMedCrossRefGoogle Scholar
  13. 13.
    Green PH, Fleischauer AT, Bhagat G, Goyal R, Jabri B, Neugut AI. Risk of malignancy in patients with celiac disease. Am J Med. 2003;115:191–5.PubMedCrossRefGoogle Scholar
  14. 14.
    Peters U, Askling J, Gridley G, Ekbom A, Linet M. Causes of death in patients with celiac disease in a population-based Swedish cohort. Arch Intern Med. 2003;163:1566–72.PubMedCrossRefGoogle Scholar
  15. 15.
    Askling J, Linet M, Gridley G, Halstensen TS, Ekstrom K, Ekbom A. Cancer incidence in a population-based cohort of individuals hospitalized with celiac disease or dermatitis herpetiformis. Gastroenterology. 2002;123:1428–35.PubMedCrossRefGoogle Scholar
  16. 16.
    Ekstrom SK, Vajdic CM, Falster M, Engels EA, Martinez-Maza O, Turner J, et al. Autoimmune disorders and risk of non-Hodgkin lymphoma subtypes: a pooled analysis within the InterLymph Consortium. Blood. 2008;111:4029–38.CrossRefGoogle Scholar
  17. 17.
    Vajdic CM, Falster MO, de Sanjose S, Martinez-Maza O, Becker N, Bracci PM, et al. Atopic disease and risk of non-Hodgkin lymphoma: an InterLymph pooled analysis. Cancer Res. 2009;69:6482–9.PubMedCrossRefGoogle Scholar
  18. 18.
    Mueller N. Overview of the epidemiology of malignancy in immune deficiency. J Acquir Immune Defic Syndr. 1999;21:S5–10.PubMedGoogle Scholar
  19. 19.
    Manns A, Cleghorn FR, Falk RT, Hanchard B, Jaffe ES, Bartholomew C, et al. Role of HTLV-I in development of non-Hodgkin lymphoma in Jamaica and Trinidad and Tobago. The HTLV Lymphoma Study Group. Lancet. 1993;342:1447–50.PubMedCrossRefGoogle Scholar
  20. 20.
    Mortreux F, Gabet AS, Wattel E. Molecular and cellular aspects of HTLV-1 associated leukemogenesis in vivo. Leukemia. 2003;17:26–38.PubMedCrossRefGoogle Scholar
  21. 21.
    Siemiatycki J, Richardson L, Boffetta P. Occupation. In: Schottenfeld D, Fraumeni JF, editors. Cancer Epidemiology and Prevention. New York: Oxford University Press; 2006. p. 322–54.CrossRefGoogle Scholar
  22. 22.
    Boffetta P, de Vocht F. Occupation and the risk of non-Hodgkin lymphoma. Cancer Epidemiol Biomarkers Prev. 2007;16:369–72.PubMedCrossRefGoogle Scholar
  23. 23.
    International Agency for Research on Cancer. IARC Monographs on the evaluation of carcinogenic risks to humans: Dry cleaning, some chlorinated solvents and other industrial chemicals. Lyon, France: International Agency for Research on Cancer;1995.Google Scholar
  24. 24.
    Engel LS, Lan Q, Rothman N. Polychlorinated biphenyls and non-Hodgkin lymphoma. Cancer Epidemiol Biomarkers Prev. 2007;16:373–6.PubMedCrossRefGoogle Scholar
  25. 25.
    Colt JS, Severson RK, Lubin J, Rothman N, Camann D, Davis S, et al. Organochlorines in carpet dust and non-Hodgkin lymphoma. Epidemiology. 2005;16:516–25.PubMedCrossRefGoogle Scholar
  26. 26.
    Colt JS, Davis S, Severson RK, Lynch CF, Cozen W, Camann D, et al. Residential insecticide use and risk of non-Hodgkin’s lymphoma. Cancer Epidemiol Biomarkers Prev. 2006;15:251–7.PubMedCrossRefGoogle Scholar
  27. 27.
    Morton LM, Hartge P, Holford TR, Holly EA, Chiu BC, Vineis P, et al. Cigarette smoking and risk of non-Hodgkin lymphoma: a pooled analysis from the International Lymphoma Epidemiology Consortium (interlymph). Cancer Epidemiol Biomarkers Prev. 2005;14:925–33.PubMedCrossRefGoogle Scholar
  28. 28.
    Besson H, Brennan P, Becker N, Nieters A, De SS, Font R, et al. Tobacco smoking, alcohol drinking and non-Hodgkin’s lymphoma: European multicenter casecontrol study (Epilymph). Int J Cancer. 2006;119:901–8.PubMedCrossRefGoogle Scholar
  29. 29.
    Morton LM, Zheng T, Holford TR, Holly EA, Chiu BC, Costantini AS, et al. Alcohol consumption and risk of non-Hodgkin lymphoma: a pooled analysis. Lancet Oncol. 2005;6:469–76.PubMedCrossRefGoogle Scholar
  30. 30.
    Gaziano JM, Gaziano TA, Glynn RJ, Sesso HD, Ajani UA, Stampfer MJ, et al. Light-to-moderate alcohol consumption and mortality in the Physicians’ Health Study enrollment cohort. J Am Coll Cardiol. 2000;35:96–105.PubMedCrossRefGoogle Scholar
  31. 31.
    Chiu BC, Cerhan JR, Gapstur SM, Sellers TA, Zheng W, Lutz CT, et al. Alcohol consumption and non-Hodgkin lymphoma in a cohort of older women. Br J Cancer. 1999;80:1476–82.PubMedCrossRefGoogle Scholar
  32. 32.
    Lim U, Morton LM, Subar AF, Baris D, Stolzenberg-Solomon R, Leitzmann M, et al. Alcohol, smoking, and body size in relation to incident Hodgkin’s and non-Hodgkin’s lymphoma risk. Am J Epidemiol. 2007;166:697–708.PubMedCrossRefGoogle Scholar
  33. 33.
    Willett EV, Morton LM, Hartge P, Becker N, Bernstein L, Boffetta P, et al. Non-Hodgkin lymphoma and obesity: a pooled analysis from the InterLymph Consortium. Int J Cancer. 2008;122:2062–70.PubMedCrossRefGoogle Scholar
  34. 34.
    Kricker A, Armstrong BK, Hughes AM, Goumas C, Smedby KE, Zheng T, et al. Personal sun exposure and risk of non Hodgkin lymphoma: a pooled analysis from the Interlymph Consortium. Int J Cancer. 2008;122:144–54.PubMedCrossRefGoogle Scholar
  35. 35.
    Zhang Y, Sanjose SD, Bracci PM, Morton LM, Wang R, Brennan P, et al. Personal use of hair dye and the risk of certain subtypes of non-Hodgkin lymphoma. Am J Epidemiol. 2008;167:1321–31.PubMedCrossRefGoogle Scholar
  36. 36.
    Rothman N, Skibola CF, Wang SS, Morgan G, Lan Q, Smith MT, et al. Genetic variation in TNF and IL10 and risk of non-Hodgkin lymphoma: a report from the InterLymph Consortium. Lancet Oncol. 2006;7:27–38.PubMedCrossRefGoogle Scholar
  37. 37.
    Skibola CF, Bracci PM, Nieters A, Brooks-Wilson A, de Sanjosé S, Hughes AM, et al. Tumor necrosis factor (TNF) and lymphotoxin-alpha (LTA) polymorphisms and risk of non-Hodgkin lymphoma in the InterLymph Consortium. Am J Epidemiol. 2010;71(3):267–76.CrossRefGoogle Scholar
  38. 38.
    Rudiger T, Weisenburger DD, Anderson JR, et al. Peripheral T-cell lymphoma (excluding anaplastic large-cell lymphoma): results from the Non-Hodgkin’s Lymphoma Classification Project. Ann Oncol. 2002;13:140–9.PubMedCrossRefGoogle Scholar
  39. 39.
    Gallamini A, Stelitano C, Calvi R, et al. Peripheral T-cell lymphoma unspecified (PTCL-U): a new prognostic model from a retrospective multicenter clinical study. Blood. 2004;103:2474–9.PubMedCrossRefGoogle Scholar
  40. 40.
    Vose JM, Armitage JO, Weisenburger DD. International peripheral T-cell lymphoma study: pathology findings and clinical outcomes. J Clin Oncol. 2008;26:4124–30.PubMedCrossRefGoogle Scholar
  41. 41.
    Siegert W, Nerl C, Agthe A, et al. Angioimmunoblastic lymphadenopathy (AILD)-type T-cell lymphoma: prognostic impact of clinical observations and laboratory findings at presentation: the Kiel Lymphoma Study Group. Ann Oncol. 1995;6:659–64.PubMedGoogle Scholar
  42. 42.
    Weisenburger DD, Gordon BG, Vose JM, et al. Occurrence of the t(2;5)(p23p35) in non-Hodgkin’s lymphoma. Blood. 1996;87:3860–8.PubMedGoogle Scholar
  43. 43.
    Jaffe ES, Chan JK, Su IJ, et al. Report of the Workshop on Nasal and Related Extranodal Angiocentric T/Natural Killer Cell Lymphomas: definitions, differential diagnosis, and epidemiology. Am J Surg Pathol. 1996;20:103–11.PubMedCrossRefGoogle Scholar
  44. 44.
    Cheung MM, Chan JK, Lau WH, et al. Primary non-Hodgkin’s lymphoma of the nose and nasopharynx: clinical features, tumor immunphenotype, and treatment outcome of 113 patients. J Clin Oncol. 1998;16:70–7.PubMedGoogle Scholar
  45. 45.
    Shimoyama M. Diagnostic criteria and classification of clinical subtypes of adult T-cell leukaemia-lymphoma: a report from the Lymphoma Study Group (1984–1987). Br J Haematol. 1991;79:428–37.PubMedCrossRefGoogle Scholar
  46. 46.
    Chen YC, Wang CH, Su IJ, et al. Infection of human T-cell leukemia virus type I and development of human T-cell leukemia lymphoma in patients with hematologic neoplasms: a possible linkage to blood transfusion. Blood. 1989;74:388–94.PubMedGoogle Scholar

Copyright information

© Springer Science+Business Media New York 2013

Authors and Affiliations

  1. 1.Department of Population SciencesCity of Hope National Medical CenterDuarteUSA
  2. 2.Section of Hematology/Oncology987680 Nebraska Medical CenterOmahaUSA

Personalised recommendations