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Peripheral T-Cell Lymphoma

  • Karin Mellgren
  • Udo KontnyEmail author
Chapter

Abstract

Non-anaplastic peripheral T-cell lymphoma (PTCL) is a heterogenous group of disorders comprising about 1% of non-Hodgkin lymphoma (NHL) in children and adolescents. Most common subtypes are peripheral T-cell lymphoma not otherwise specified (PTCL-NOS) followed by NK−/T-cell lymphoma (NKTCL), hepatosplenic T-cell lymphoma (HSTCL), and subcutaneous panniculitis-like T-cell lymphoma (SPLTCL). Prognosis of PTCL in children and adolescents is worse than for other childhood NHLs with 5-y EFS around 50% and 5-y OS around 55% but still better than in adults. Survival rates are subtype-specific and best for SPLTCL, intermediate for PTCL-NOS and NKTCL, and worse for HSTCL, requiring subtype-oriented treatment approaches.

Keywords

Non-anaplastic peripheral T-cell lymphoma Peripheral T-cell lymphoma not otherwise specified Hepatosplenic T-cell lymphoma Subcutaneous panniculitis T-cell lymphoma Angioimmunoblastic T-cell lymphoma 

Notes

Acknowledgment

We thank Wolfram Klapper, M.D. and Rex K.H. Au-Yeung, M.D. for providing the histological pictures.

References

  1. 1.
    Kontny U, Oschlies I, Woessmann W, Burkhardt B, Lisfeld J, Salzburg J, Janda A, Attarbaschi A, Niggli F, Zimmermann M, Reiter A, Klapper W. Non-anaplastic peripheral T-cell lymphomas in children and adolescents – a retrospective analysis of the NHL-BFM study group. Br J Haematol. 2015;168(6):835–44.CrossRefGoogle Scholar
  2. 2.
    Windsor R, Stiller C, Webb D. Peripheral T-cell lymphoma in childhood: population-based experience in the United Kingdom over 20 years. Pediatr Blood Cancer. 2008;50(4):784–7.CrossRefGoogle Scholar
  3. 3.
    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
  4. 4.
    Swerdlow SH, Campo E, Pileri SA, Harris NL, Stein H, Siebert R, Advani R, Ghielmini M, Salles GA, Zelenetz AD, Jaffe ES. The 2016 revision of the World Health Organization classification of lymphoid neoplasms. Blood. 2016;127(20):2375–90.CrossRefGoogle Scholar
  5. 5.
    William BM, Armitage JO. International analysis of the frequency and outcome of NK/T-cell lymphomas. Best Pract Res Clin Haematol. 2013;26(1):23–32.CrossRefGoogle Scholar
  6. 6.
    Vose J, Armitage J, Weisenburger D, International T-Cell Lymphoma Project. International peripheral T-cell and natural killer/T-cell lymphoma study: pathology findings and clinical outcomes. J Clin Oncol. 2008;26(25):4124–30.CrossRefGoogle Scholar
  7. 7.
    Mellgren K, Attarbaschi A, Abla O, Alexander S, Bomken S, Bubanska E, Chiang A, Csóka M, Fedorova A, Kabickova E, Kobayashi R, Krenova Z, Meyer-Wentrup F, Miakova N, Pillon M, Plat G, Uyttebroeck A, Williams D, Wróbel G, Kontny U, on behalf of the European Intergroup for Childhood Non-Hodgkin Lymphoma (EICNHL) and the international Berlin-Frankfurt-Münster (i-BFM) Group. Non-anaplastic peripheral T-cell lymphoma in children and adolescents – an international review of 143 cases. Ann Hematol. 2016;95(8):1295–305.CrossRefGoogle Scholar
  8. 8.
    Hutchinson RE, Laver JH, Chang M, Muzzafar T, Desai S, Murphy S, Schwenn M, Shuster J, Link MP. Non-anaplastic peripheral T-cell lymphoma in childhood and adolescence: a Children’s Oncology Group Study. Pediatr Blood Cancer. 2008;51:29–33.CrossRefGoogle Scholar
  9. 9.
    Kobayashi R, Yamato K, Tanaka F, Takashima Y, Inada H, Kikuchi A, Kumagai MA, Sunami S, Nakagawa A, Fukano R, Fujita N, Mitsui T, Tsurusawa M, Mori T, Lymphoma Committee, Japanese Pediatric Leukemia/Lymphoma Study Group. Retrospective analysis of non-anaplastic peripheral T-cell lymphoma in pediatric patients in Japan. Pediatr Blood Cancer. 2010;54(2):212–5.PubMedPubMedCentralGoogle Scholar
  10. 10.
    Al Mahmoud R, Weitzman S, Schechter T, Ngan B, Abdelhaleem M, Alexander S. Peripheral T-cell lymphomas in children and adolescents: a single-institution experience. J Pediatr Hematol Oncol. 2012;34:611–6.CrossRefGoogle Scholar
  11. 11.
    Wang ZY, Li YX, Wang WH, Jin J, Wang H, Song YW, et al. Primary radiotherapy showed favorable outcome in treating extranodal nasal-type NK/T-cell lymphoma in children and adolescents. Blood. 2009;114(23):4771–6.CrossRefGoogle Scholar
  12. 12.
    Langerak AW, Groenen PJ, Bruggemann M, Beldjord K, Bellan C, Bonello L, et al. EuroClonality/BIOMED-2 guidelines for interpretation and reporting of Ig/TCR clonality testing in suspected lymphoproliferations. Leukemia. 2012;26(10):2159–71.CrossRefGoogle Scholar
  13. 13.
    Sandlund JT, Perkins SL. Uncommon non-Hodgkin lymphomas of childhood: pathological diagnosis, clinical features and treatment approaches. Br J Haematol. 2015;169(5):631–46.CrossRefGoogle Scholar
  14. 14.
    Ellin F, Landström J, Jerkeman M, Relander T. Real-world data on prognostic factors and treatment in peripheral T-cell lymphomas: a study from the Swedish Lymphoma registry. Blood. 2014;124(10):1570–7.CrossRefGoogle Scholar
  15. 15.
    Ballester B, Ramuz O, Gisselbrecht C, et al. Gene expression profiling identifies molecular subgroups among nodal peripheral T-cell lymphomas. Oncogene. 2006;25(10):1560–70.CrossRefGoogle Scholar
  16. 16.
    Sakata-Yanagimoto M, Chiba S. Molecular pathogenesis of peripheral T cell lymphoma. Curr Hematol Malig Rep. 2015;10:429–37.CrossRefGoogle Scholar
  17. 17.
    Iqbal J, Wright G, Wang C, Rosenwald A, Gascoyne RD, Weisenburger DD, Greiner TC, Smith L, Guo S, Wilcox RA, Teh BT, Lim ST, Tan SY, Rimsza LM, Jaffe ES, Campo E, Martinez A, Delabie J, Braziel RM, Cook JR, Tubbs RR, Ott G, Geissinger E, Gaulard P, Piccaluga PP, Pileri SA, Au WY, Nakamura S, Seto M, Berger F, de Leval L, Connors JM, Armitage J, Vose J, Chan WC, Staudt LM, Lymphoma Leukemia Molecular Profiling Project and the International Peripheral T-cell Lymphoma Project. Gene expression signatures delineate biological and prognostic subgroups in peripheral T-cell lymphoma. Blood. 2014;123(19):2915–23.CrossRefGoogle Scholar
  18. 18.
    Palomero T, Couronne L, Khiabanian H, et al. Recurrent mutations in epigenetic regulators, RHOA and FYN kinase in peripheral T cell lymphomas. Nat Genet. 2014;46(2):166–70.CrossRefGoogle Scholar
  19. 19.
    Yoo HY, Sung MK, Lee SH, et al. A recurrent inactivating mutation in RHOA GTPase in angioimmunoblastic T cell lymphoma. Nat Genet. 2014;46(4):371–5.CrossRefGoogle Scholar
  20. 20.
    Couronne L, Bastard C, Bernard OA. TET2 and DNMT3A mutations in human T-cell lymphoma. N Engl J Med. 2012;366(1):95–6.CrossRefGoogle Scholar
  21. 21.
    Streubel B, Vinatzer U, Willheim M, Raderer M, Chott A. Novel t(5;9)(q33;q22) fuses ITK to SYK in unspecified peripheral T-cell lymphoma. Leukemia. 2006;20(2):313–8.CrossRefGoogle Scholar
  22. 22.
    Fujiwara SI, Yamashita Y, Nakamura N, et al. High-resolution analysis of chromosome copy number alterations in angioimmunoblastic T-cell lymphoma and peripheral T-cell lymphoma, unspecified, with single nucleotide polymorphism-typing microarrays. Leukemia. 2008;22(10):1891–8.CrossRefGoogle Scholar
  23. 23.
    Sandell RF, Boddicker RL, Feldman AL. Genetic landscape and classification of peripheral T cell lymphomas. Curr Oncol Rep. 2017;19:28.CrossRefGoogle Scholar
  24. 24.
    Nelson M, Horsman DE, Weisenburger DD, Gascoyne RD, Dave BJ, Loberiza FR, Ludkovski O, Savage KJ, Armitage JO, Sanger WG. Cytogenetic abnormalities and clinical correlations in peripheral T-cell lymphoma. Br J Haematol. 2008;141(4):461–9.CrossRefGoogle Scholar
  25. 25.
    Seidemann K, Tiemann M, Schrappe M, Yakisan E, Simonitsch I, Janka-Schaub G, et al. Short-pulse B-non-Hodgkin lymphoma-type chemotherapy is efficacious treatment for pediatric anaplastic large cell lymphoma: a report of the Berlin-Frankfurt-Munster Group Trial NHL-BFM 90. Blood. 2001;97(12):3699–706.CrossRefGoogle Scholar
  26. 26.
    Kobayashi R, Fujita N, Mitsui T, Iwasaki F, Suzumiya J, Kuroda H, et al. Stem cell transplantation for paediatric patients with non-anaplastic peripheral T-cell lymphoma in Japan. Br J Haematol. 2012;159(1):88–93.CrossRefGoogle Scholar
  27. 27.
    Casulo C, Schoder H, Feeney J, Lim R, Maragulia J, Zelenetz AD, et al. 18F-fluorodeoxyglucose positron emission tomography in the staging and prognosis of T cell lymphoma. Leuk Lymphoma. 2013;54(10):2163–7.CrossRefGoogle Scholar
  28. 28.
    Attygalle AD, Cabecadas J, Gaulard P, Jaffe E, de Jong D, Hyeh Ko Y, Said J, Klapper W. Peripheral T-cell and NK-cell lymphomas and their mimics; taking a step forward – report on the lymphoma workshop of the XVIth meeting of the European Association for Haematopathology and the Society for Hematopathology. Histopathology. 2014;64:171–99.CrossRefGoogle Scholar
  29. 29.
    Macon WR, Levy NB, Kurtin PJ, Salhany KE, Elkhalifa MY, Casey TT, Craig FE, Vnencak-Jones CL, Gulley ML, Park JP, Cousar JB. Hepatosplenic alphabeta T-cell lymphomas: a report of 14 cases and comparison with hepatosplenic gammadelta T-cell lymphomas. Am J Surg Pathol. 2001;25(3):285–96.CrossRefGoogle Scholar
  30. 30.
    Belhadj K, Reyes F, Farcet JP, Tilly H, Bastard C, Angonin R, et al. Hepatosplenic gammadelta T-cell lymphoma is a rare clinicopathologic entity with poor outcome: report on a series of 21 patients. Blood. 2003;102(13):4261–9.CrossRefGoogle Scholar
  31. 31.
    Willemze R. Thirty years of progress in cutaneous lymphoma research. G Ital Dermatol Venereol. 2012;147(6):515–21.PubMedGoogle Scholar
  32. 32.
    Oschlies I, Simonitsch-Klupp I, Maldyk J, Konovalov D, Abramov D, Myakova N, Lisfeld J, Attarbaschi A, Kontny U, Woessmann W, Klapper W. Subcutaneous panniculitis-like T-cell lymphoma in children: a detailed clinicopathological description of 11 multifocal cases with a high frequency of haemophagocytic syndrome. Br J Dermatol. 2015;172(3):793–7.CrossRefGoogle Scholar
  33. 33.
    Sakurai E, et al. Subcutaneous Panniculitis-Like T-Cell Lymphoma (SPTCL) with Hemophagocytosis (HPS): Successful Treatment Using High-Dose Chemotherapy (BFM-NHL & ALL-90) and Autologous Peripheral Blood Stem Cell Transplantation. J Clin Exp Hematop. 2013;53(2):135–40.CrossRefGoogle Scholar
  34. 34.
    Iannitto E, Ferreri AJ, Minardi V, Tripodo C, Kreipe HH. Angioimmunoblastic T-cell lym- phoma. Crit Rev Oncol Hematol. 2008;68:264–71.CrossRefGoogle Scholar
  35. 35.
    Kraus T, Twist CJ, Tan BT. Angioimmunoblastic T cell lymphoma: an unusual presentation of posttransplant lymphoproliferative disorder in a pediatric patient. Acta Haematol. 2014;131:95–101.CrossRefGoogle Scholar
  36. 36.
    de Leval L, Rickman DS, Thielen C, et al. The gene expression profile of nodal peripheral T-cell lymphoma demonstrates a molecular link between angioimmunoblastic T-cell lymphoma (AITL) and follicular helper T (TFH) cells. Blood. 2007;109(11):4952–63.CrossRefGoogle Scholar
  37. 37.
    Piccaluga PP, Agostinelli C, Califano A, et al. Gene expression analysis of angioimmunoblastic lymphoma indicates derivation from T follicular helper cells and vascular endothelial growth fac- tor deregulation. Cancer Res. 2007;67(22):10703–10.CrossRefGoogle Scholar
  38. 38.
    Iqbal J, Weisenburger DD, Greiner TC, Vose JM, McKeithan T, Kucuk C, Geng H, Deffenbacher K, Smith L, Dybkaer K, Nakamura S, Seto M, Delabie J, Berger F, Loong F, Au WY, Ko YH, Sng I, Armitage JO, Chan WC, International Peripheral T-Cell Lymphoma Project. Molecular signatures to improve diagnosis in peripheral T-cell lymphoma and prognostication in angioimmunoblastic T-cell lymphoma. Blood. 2010;115(5):1026–36.CrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Department of Pediatric Hematology and OncologyThe Queen Silvia’s Hospital for ChildrenGöteborgSweden
  2. 2.Division of Pediatric Hematology, Oncology and Stem Cell Transplantation, Department of Pediatrics and Adolescent MedicineUniversity Medical Center AachenAachenGermany

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