Skip to main content
SpringerLink
Log in

Peripheral T-Cell Lymphoma, not Otherwise Specified (PTCL-NOS)

  • Chapter
  • First Online:
T-Cell and NK-Cell Lymphomas

Part of the book series: Cancer Treatment and Research ((CTAR,volume 176))

Abstract

Peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS) is a World Health Organization (WHO)-defined diagnostic category within the highly heterogeneous group of mature post-thymic T-cell neoplasms. It is the most common subtype of mature post-thymic T-cell neoplasms globally, accounting for up to 35% of PTCL cases in Europe and North America. PTCL-NOS is a diagnosis of exclusion, comprising several disease entities that differ in biology, clinical presentation, and outcome. The diagnosis of PTCL-NOS is made based on the presence of typical histopathological features of lymphoma, an aberrant T-cell immunophenotype, often with a loss of CD5 and CD7, and a clonal T-cell receptor (TCR) gene rearrangement, in the appropriate clinical context. Unlike other types of T-cell lymphoma, recurrent mutations to assist with the diagnosis have not been identified. Patients often present with advanced stage. Prognosis is poor, with a 5-year overall survival (OS) of 20-30%. Anthracycline-based combination chemotherapy remains the most frequently used frontline strategy, with overall response rates (ORR) of 50-60%, and complete response rates (CRR) of 20-30%. Prospective studies with intent-to-treat analyses have shown that consolidation with high-dose chemotherapy and autologous stem cell transplant (ASCT) results in progression-free survivals (PFS) that compare favorably with historical cohorts and may improve OS in selected patient populations. However, randomized data are still lacking. Over the past decade, therapeutic agents approved in the relapsed and refractory setting have produced response rates of up to 33% and median PFS up to 18 months. Overall, outcomes remain poor and there is a dire need for more effective treatments. This review discusses the latest information on the diagnosis and treatment of PTCL-NOS.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 129.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Hardcover Book
USD 169.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Broccoli A, Zinzani PL (2017) Peripheral T-cell lymphoma, not otherwise specified. Blood 129(9):1103–1112

    Article  CAS  Google Scholar 

  2. Carson KR et al (2017) A prospective cohort study of patients with peripheral T-cell lymphoma in the United States. Cancer 123(7):1174–1183

    Article  Google Scholar 

  3. Foss FM et al (2011) Peripheral T-cell lymphoma. Blood 117(25):6756–6767

    Article  CAS  Google Scholar 

  4. Vose J et al (2008) International peripheral T-cell and natural killer/T-cell lymphoma study: pathology findings and clinical outcomes. J Clin Oncol 26(25):4124–4130

    Article  Google Scholar 

  5. Vose JM (2008) Peripheral T-cell non-Hodgkin’s lymphoma. Hematol Oncol Clin North Am 22(5): 997–1005, x

    Article  Google Scholar 

  6. Armitage JO (2015) The aggressive peripheral T-cell lymphomas: 2015. Am J Hematol 90(7):665–673

    Article  Google Scholar 

  7. Dhawale TM, Shustov AR (2017) Autologous and allogeneic hematopoietic cell transplantation in peripheral T/NK-cell lymphomas: a histology-specific review. Hematol Oncol Clin North Am 31(2):335–357

    Article  Google Scholar 

  8. Horwitz SM et al (2016) NCCN guidelines insights: non-Hodgkin’s lymphomas, version 3.2016. J Natl Compr Cancer Netw 14(9):1067–1079

    Article  Google Scholar 

  9. Bellei M et al (2015) The value and relevance of the T cell lymphoma registries and international collaborations: the case of COMPLETE and the T-cell project. Curr Hematol Malig Rep 10(4):448–455

    Article  Google Scholar 

  10. Hildyard C et al (2017) Toward a biology-driven treatment strategy for peripheral T-cell lymphoma. Clin Med Insights Blood Disord 10:1179545X17705863

    Google Scholar 

  11. Adams SV, Newcomb PA, Shustov AR (2016) Racial patterns of peripheral T-cell lymphoma incidence and survival in the United States. J Clin Oncol 34(9):963–971

    Article  CAS  Google Scholar 

  12. Weisenburger DD et al (2011) Peripheral T-cell lymphoma, not otherwise specified: a report of 340 cases from the international peripheral T-cell lymphoma project. Blood 117(12):3402–3408

    Article  CAS  Google Scholar 

  13. Phan A, Veldman R, Lechowicz MJ (2016) T-cell lymphoma epidemiology: the known and unknown. Curr Hematol Malig Rep 11(6):492–503

    Article  Google Scholar 

  14. Wang SS et al (2014) 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(48):66–75

    Article  Google Scholar 

  15. Swerdlow SH et al (2016) The 2016 revision of the World Health Organization classification of lymphoid neoplasms. Blood 127(20):2375–2390

    Article  CAS  Google Scholar 

  16. Al-Zahrani M, Savage KJ (2017) Peripheral T-cell lymphoma, not otherwise specified: a review of current disease understanding and therapeutic approaches. Hematol Oncol Clin North Am 31(2):189–207

    Article  Google Scholar 

  17. Casulo C et al (2017) T-cell lymphoma: recent advances in characterization and new opportunities for treatment. J Natl Cancer Inst 109(2)

    Article  Google Scholar 

  18. Leonard JP, Martin P, Roboz GJ (2017) Practical implications of the 2016 revision of the World Health Organization classification of lymphoid and myeloid neoplasms and acute leukemia. J Clin Oncol 35(23):2708–2715

    Article  CAS  Google Scholar 

  19. Schmitz N, de Leval L (2017) How I manage peripheral T-cell lymphoma, not otherwise specified and angioimmunoblastic T-cell lymphoma: current practice and a glimpse into the future. Br J Haematol 176(6):851–866

    Article  CAS  Google Scholar 

  20. Maura F (2016) Biology of peripheral T cell lymphomas—not otherwise specified: is something finally happening? Pathogenesis 3(1):9–18

    Article  Google Scholar 

  21. Savage KJ et al (2011) Peripheral T-cell lymphoma–not otherwise specified. Crit Rev Oncol Hematol 79(3):321–329

    Article  Google Scholar 

  22. Yoshida N et al (2013) Primary peripheral T-cell lymphoma, not otherwise specified of the thyroid with autoimmune thyroiditis. Br J Haematol 161(2):214–223

    Article  CAS  Google Scholar 

  23. Geissinger E et al (2009) CD52 expression in peripheral T-cell lymphomas determined by combined immunophenotyping using tumor cell specific T-cell receptor antibodies. Leuk Lymphoma 50(6):1010–1016

    Article  CAS  Google Scholar 

  24. Jiang L et al (2009) Variable CD52 expression in mature T cell and NK cell malignancies: implications for alemtuzumab therapy. Br J Haematol 145(2):173–179

    Article  CAS  Google Scholar 

  25. Hsi ED et al (2017) Analysis of peripheral T-cell lymphoma diagnostic workup in the United States. Clin Lymphoma Myeloma Leuk 17(4):193–200

    Article  Google Scholar 

  26. Sakata-Yanagimoto M, Chiba S (2015) Molecular pathogenesis of peripheral T cell lymphoma. Curr Hematol Malig Rep 10(4):429–437

    Article  Google Scholar 

  27. Iqbal J et al (2014) Gene expression signatures delineate biological and prognostic subgroups in peripheral T-cell lymphoma. Blood 123(19):2915–2923

    Article  CAS  Google Scholar 

  28. de Leval L, Gaulard P (2014) Cellular origin of T-cell lymphomas. Blood 123(19):2909–2910

    Article  Google Scholar 

  29. de Leval L et al (2007) 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 109(11):4952–4963

    Article  Google Scholar 

  30. Xu P et al (2015) Analysis of prognostic factors and comparison of prognostic scores in peripheral T cell lymphoma, not otherwise specified: a single-institution study of 105 Chinese patients. Ann Hematol 94(2):239–247

    Article  CAS  Google Scholar 

  31. Gutierrez-Garcia G et al (2011) Comparison of four prognostic scores in peripheral T-cell lymphoma. Ann Oncol 22(2):397–404

    Article  CAS  Google Scholar 

  32. Ellin F et al (2014) Real-world data on prognostic factors and treatment in peripheral T-cell lymphomas: a study from the Swedish Lymphoma Registry. Blood 124(10):1570–1577

    Article  CAS  Google Scholar 

  33. Haverkos BM et al (2017) Frequency and clinical correlates of elevated plasma Epstein-Barr virus DNA at diagnosis in peripheral T-cell lymphomas. Int J Cancer 140(8):1899–1906

    Article  CAS  Google Scholar 

  34. Gru AA et al (2015) The Epstein-Barr Virus (EBV) in T cell and NK cell lymphomas: time for a reassessment. Curr Hematol Malig Rep 10(4):456–467

    Article  CAS  Google Scholar 

  35. Briski R et al (2014) The role of front-line anthracycline-containing chemotherapy regimens in peripheral T-cell lymphomas. Blood Cancer J 4:e214

    Article  CAS  Google Scholar 

  36. Abouyabis AN et al (2011) A systematic review and meta-analysis of front-line anthracycline-based chemotherapy regimens for peripheral T-cell lymphoma. ISRN Hematol 2011:623924

    Article  Google Scholar 

  37. Schmitz N et al (2010) Treatment and prognosis of mature T-cell and NK-cell lymphoma: an analysis of patients with T-cell lymphoma treated in studies of the German High-Grade Non-Hodgkin Lymphoma Study Group. Blood 116(18):3418–3425

    Article  CAS  Google Scholar 

  38. Kim JG et al (2006) CHOP plus etoposide and gemcitabine (CHOP-EG) as front-line chemotherapy for patients with peripheral T cell lymphomas. Cancer Chemother Pharmacol 58(1):35–39

    Article  Google Scholar 

  39. Mahadevan D et al (2013) Phase 2 trial of combined cisplatin, etoposide, gemcitabine, and methylprednisolone (PEGS) in peripheral T-cell non-Hodgkin lymphoma: Southwest Oncology Group Study S0350. Cancer 119(2):371–379

    Article  CAS  Google Scholar 

  40. Advani RH et al (2016) A phase II study of cyclophosphamide, etoposide, vincristine and prednisone (CEOP) Alternating with Pralatrexate (P) as front line therapy for patients with peripheral T-cell lymphoma (PTCL): final results from the T-cell consortium trial. Br J Haematol 172(4):535–544

    Article  CAS  Google Scholar 

  41. Simon A et al (2010) Upfront VIP-reinforced-ABVD (VIP-rABVD) is not superior to CHOP/21 in newly diagnosed peripheral T cell lymphoma. Results of the randomized phase III trial GOELAMS-LTP95. Br J Haematol 151(2):159–166

    Article  Google Scholar 

  42. Escalon MP et al (2005) Prognostic factors and treatment of patients with T-cell non-Hodgkin lymphoma: the M. D. Anderson Cancer Center experience. Cancer 103(10):2091–2098

    Article  Google Scholar 

  43. Mehta N et al (2013) A retrospective analysis of peripheral T-cell lymphoma treated with the intention to transplant in the first remission. Clin Lymphoma Myeloma Leuk 13(6):664–670

    Article  Google Scholar 

  44. Beaven AW, Diehl LF (2015) Peripheral T-cell lymphoma, NOS, and anaplastic large cell lymphoma. Hematology Am Soc Hematol Educ Program 2015:550–558

    Article  Google Scholar 

  45. Smith SM et al (2013) Hematopoietic cell transplantation for systemic mature T-cell non-Hodgkin lymphoma. J Clin Oncol 31(25):3100–3109

    Article  Google Scholar 

  46. d’Amore F et al (2012) Up-front autologous stem-cell transplantation in peripheral T-cell lymphoma: NLG-T-01. J Clin Oncol 30(25):3093–3099

    Article  Google Scholar 

  47. Feyler S et al (2007) The role of high-dose therapy and stem cell rescue in the management of T-cell malignant lymphomas: a BSBMT and ABMTRR study. Bone Marrow Transpl 40(5):443–450

    Article  CAS  Google Scholar 

  48. Abramson JS et al (2014) Peripheral T-cell lymphomas in a large US multicenter cohort: prognostication in the modern era including impact of frontline therapy. Ann Oncol 25(11):2211–2217

    Article  CAS  Google Scholar 

  49. Rodriguez J et al (2003) High-dose chemotherapy and autologous stem cell transplantation in peripheral T-cell lymphoma: the GEL-TAMO experience. Ann Oncol 14(12):1768–1775

    Article  CAS  Google Scholar 

  50. Rodriguez J et al (2003) High dose chemotherapy and autologous stem cell transplantation in patients with peripheral T-cell lymphoma not achieving complete response after induction chemotherapy. The GEL-TAMO experience. Haematologica 88(12):1372–1377

    CAS  PubMed  Google Scholar 

  51. Yang DH et al (2009) Prognostic factors and clinical outcomes of high-dose chemotherapy followed by autologous stem cell transplantation in patients with peripheral T cell lymphoma, unspecified: complete remission at transplantation and the prognostic index of peripheral T cell lymphoma are the major factors predictive of outcome. Biol Blood Marrow Transpl 15(1):118–125

    Article  CAS  Google Scholar 

  52. Corradini P et al (2014) Intensified chemo-immunotherapy with or without stem cell transplantation in newly diagnosed patients with peripheral T-cell lymphoma. Leukemia 28(9):1885–1891

    Article  CAS  Google Scholar 

  53. Schmitz N et al (2015) Allogeneic or autologous transplantation as first-line therapy for younger patients with peripheral T-cell lymphoma: results of the interim analysis of the AATT trial. J Clin Oncol 33(15): 8507

    Google Scholar 

  54. Beitinjaneh A et al (2015) Comparison of survival in patients with T cell lymphoma after autologous and allogeneic stem cell transplantation as a frontline strategy or in relapsed disease. Biol Blood Marrow Transpl 21(5):855–859

    Article  Google Scholar 

  55. Hosing C, Champlin RE (2011) Stem-cell transplantation in T-cell non-Hodgkin’s lymphomas. Ann Oncol 22(7):1471–1477

    Article  CAS  Google Scholar 

  56. Song KW et al (2003) Autologous stem cell transplant for relapsed and refractory peripheral T-cell lymphoma: variable outcome according to pathological subtype. Br J Haematol 120(6):978–985

    Article  Google Scholar 

  57. Moskowitz AJ, Lunning MA, Horwitz SM (2014) How I treat the peripheral T-cell lymphomas. Blood 123(17):2636–2644

    Article  CAS  Google Scholar 

  58. Corradini P et al (2004) Graft-versus-lymphoma effect in relapsed peripheral T-cell non-Hodgkin’s lymphomas after reduced-intensity conditioning followed by allogeneic transplantation of hematopoietic cells. J Clin Oncol 22(11):2172–2176

    Article  Google Scholar 

  59. Mak V et al (2013) Survival of patients with peripheral T-cell lymphoma after first relapse or progression: spectrum of disease and rare long-term survivors. J Clin Oncol 31(16):1970–1976

    Article  CAS  Google Scholar 

  60. Marchi E, Raufi AG, O’Connor OA (2017) Novel agents in the treatment of relapsed or refractory peripheral T-cell lymphoma. Hematol Oncol Clin North Am 31(2):359–375

    Article  Google Scholar 

  61. Zinzani PL et al (2010) Gemcitabine as single agent in pretreated T-cell lymphoma patients: evaluation of the long-term outcome. Ann Oncol 21(4):860–863

    Article  CAS  Google Scholar 

  62. Dreyling M et al (2013) ESMO consensus conferences: guidelines on malignant lymphoma. part 2: marginal zone lymphoma, mantle cell lymphoma, peripheral T-cell lymphoma. Ann Oncol 24(4):857–877

    Article  CAS  Google Scholar 

  63. Shi Y et al (2015) Results from a multicenter, open-label, pivotal phase II study of chidamide in relapsed or refractory peripheral T-cell lymphoma. Ann Oncol 26(8):1766–1771

    Article  CAS  Google Scholar 

  64. Marchi E, O’Connor OA (2012) Safety and efficacy of pralatrexate in the treatment of patients with relapsed or refractory peripheral T-cell lymphoma. Ther Adv Hematol 3(4):227–235

    Article  CAS  Google Scholar 

  65. Wood GS, Wu J (2015) Methotrexate and pralatrexate. Dermatol Clin 33(4):747–755

    Article  CAS  Google Scholar 

  66. Bose P, Dai Y, Grant S (2014) Histone deacetylase inhibitor (HDACI) mechanisms of action: emerging insights. Pharmacol Ther 143(3):323–336

    Article  CAS  Google Scholar 

  67. van de Donk NW, Dhimolea E (2012) Brentuximab vedotin. MAbs 4(4):458–465

    Article  Google Scholar 

  68. Briski R et al (2015) Survival in patients with limited-stage peripheral T-cell lymphomas. Leuk Lymphoma 56(6):1665–1670

    Article  Google Scholar 

  69. Gurion R et al (2016) Central nervous system involvement in T-cell lymphoma: a single center experience. Acta Oncol 55(5):561–566

    Article  CAS  Google Scholar 

  70. Pro B, Perini G (2010) Central nervous system prophylaxis in peripheral T-cell lymphoma. Blood 115(26):5427

    Article  CAS  Google Scholar 

  71. Park HS et al (2017) T-cell non-Hodgkin lymphomas: spectrum of disease and the role of imaging in the management of common subtypes. Korean J Radiol 18(1):71–83

    Article  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Division of Hematologic Malignancies and Hematopoietic Stem Cell Transplantation, Department of Medical Oncology, Sidney Kimmel Cancer Center, Thomas Jefferson University, 834 Chestnut Street, Suite 320, Philadelphia, PA, 19107, USA

    Adetokunbo Oluwasanjo, Saritha Kartan, William Johnson & Onder Alpdogan

  2. Department of Pathology, Thomas Jefferson University, Philadelphia, USA

    Jerald Gong

  3. Department of Medicine, Comprehensive Cancer Center, The Ohio State University, Columbus, USA

    Anjali Mishra

  4. Department of Pathology, University of Virginia, Charlottesville, USA

    Alejandro Gru

  5. Division of Hematology, University of Colorado, Denver, USA

    Bradley M. Haverkos & Pierluigi Porcu

Authors
  1. Adetokunbo Oluwasanjo
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Saritha Kartan
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. William Johnson
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Onder Alpdogan
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Alejandro Gru
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Anjali Mishra
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Bradley M. Haverkos
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Jerald Gong
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Pierluigi Porcu
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Pierluigi Porcu .

Editor information

Editors and Affiliations

  1. Division of Dermatology, Beckman Research Institute, City of Hope National Medical Center, Duarte, CA, USA

    Christiane Querfeld

  2. Department of Hematology and Hematopoietic Cell Transplantation, City of Hope National Medical Center, Duarte, CA, USA

    Jasmine Zain

  3. Department of Hematology and Hematopoietic Cell Transplantation, Beckman Research Institute, City of Hope National Medical Center, Duarte, CA, USA

    Steven T. Rosen

Rights and permissions

Reprints and permissions

Copyright information

© 2019 Springer Nature Switzerland AG

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Oluwasanjo, A. et al. (2019). Peripheral T-Cell Lymphoma, not Otherwise Specified (PTCL-NOS). In: Querfeld, C., Zain, J., Rosen, S. (eds) T-Cell and NK-Cell Lymphomas. Cancer Treatment and Research, vol 176. Springer, Cham. https://doi.org/10.1007/978-3-319-99716-2_4

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-99716-2_4

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-99715-5

  • Online ISBN: 978-3-319-99716-2

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation