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Clinical Management of Non-cutaneous T-cell and NK-cell Malignancies

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Neoplastic Hematopathology

Part of the book series: Contemporary Hematology ((CH))

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Abstract/Scope of Chapter

The uncommon but heterogeneous group of peripheral T-cell lymphomas and leukemias are now recognized to be derived from various functional subsets of post-thymic (mature) T cells. Conventional multi-agent chemotherapeutic regimens, developed primarily for B-cell tumors, generally work poorly for T-cell tumors. In this chapter, we outline the current clinical approaches to staging, outcome prediction, and therapy in T-cell and NK-cell malignancies presenting in the lymph node, peripheral blood, bone marrow and at extranodal sites with the exception of cutaneous T-cell lymphomas and Sézary syndrome which are covered in Chap. 25. We describe frontline and salvage therapies, introduce prognostic models, and evaluate the role of autologous and allogeneic stem cell transplantation. Finally, an overview of novel T-cell selective therapeutic agents is presented.

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References

  1. Armitage J, Vose J, Weisenburger D. 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 

  2. Dunleavy K, Wilson WH, Jaffe ES. Angioimmunoblastic T cell lymphoma: pathobiological insights and clinical implications. Curr Opin Hematol 2007;14(4):348-53.

    Article  PubMed  CAS  Google Scholar 

  3. Savage KJ, Harris NL, Vose JM, et al. ALK- anaplastic large-cell lymphoma is clinically and immunophenotypically different from both ALK+ ALCL and peripheral T-cell lymphoma, not otherwise specified: report from the International Peripheral T-Cell Lymphoma Project. Blood 2008;111(12):5496-504.

    Article  PubMed  CAS  Google Scholar 

  4. Piccaluga PP, Agostinelli C, Califano A, et al. Gene expression analysis of peripheral T cell lymphoma, unspecified, reveals distinct profiles and new potential therapeutic targets. J Clin Invest 2007;117(3):823-34.

    Article  PubMed  CAS  Google Scholar 

  5. 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.

    Article  PubMed  Google Scholar 

  6. Rodriguez-Abreu D, Filho VB, Zucca E. Peripheral T-cell lymphomas, unspecified (or not otherwise specified): a review. Hematol Oncol 2008;26(1):8-20.

    Article  PubMed  CAS  Google Scholar 

  7. Lee Y, Uhm JE, Lee HY, et al. Clinical features and prognostic factors of patients with “peripheral T cell lymphoma, unspecified”. Ann Hematol 2009;88(2):111-9.

    Article  PubMed  Google Scholar 

  8. Agostinelli C, Piccaluga PP, Went P, et al. Peripheral T cell lymphoma, not otherwise specified: the stuff of genes, dreams and therapies. J Clin Pathol 2008;61(11):1160-7.

    Article  PubMed  CAS  Google Scholar 

  9. Zettl A, deLeeuw R, Haralambieva E, Mueller-Hermelink HK. Enteropathy-type T-cell lymphoma. Am J Clin Pathol 2007;127(5):701-6.

    Article  PubMed  Google Scholar 

  10. Weidmann E. Hepatosplenic T cell lymphoma. A review on 45 cases since the first report describing the disease as a distinct lymphoma entity in 1990. Leukemia 2000;14(6):991-7.

    Article  PubMed  CAS  Google Scholar 

  11. Herling M, Khoury JD, Washington LT, Duvic M, Keating MJ, Jones D. A systematic approach to diagnosis of mature T-cell leukemias reveals heterogeneity among WHO categories. Blood 2004;104(2):328-35.

    Article  PubMed  CAS  Google Scholar 

  12. Herling M, Patel KA, Teitell MA, et al. High TCL1 expression and intact T-cell receptor signaling define a hyperproliferative subset of T-cell prolymphocytic leukemia. Blood 2008;111(1):328-37.

    Article  PubMed  CAS  Google Scholar 

  13. Matutes E. Adult T-cell leukaemia/lymphoma. J Clin Pathol 2007;60(12):1373-7.

    Article  PubMed  CAS  Google Scholar 

  14. Biggar RJ, Engels EA, Frisch M, Goedert JJ. Risk of T-cell lymphomas in persons with AIDS. J Acquir Immune Defic Syndr 1999, 2001;26(4):371-6.

    Google Scholar 

  15. Jamali FR, Otrock ZK, Soweid AM, et al. An overview of the pathogenesis and natural history of post-transplant T-cell lymphoma (corrected and republished article originally printed in Leukemia & Lymphoma, June 2007; 48(6): 1237-1241). Leuk Lymphoma 2007;48(9):1780-4.

    Article  PubMed  Google Scholar 

  16. Liang X, Graham DK. Natural killer cell neoplasms. Cancer 2008;112(7):1425-36.

    Article  PubMed  Google Scholar 

  17. Kim TM, Lee SY, Jeon YK, et al. Clinical heterogeneity of extranodal NK/T-cell lymphoma, nasal type: a national survey of the Korean Cancer Study Group. Ann Oncol 2008;19(8):1477-84.

    Article  PubMed  CAS  Google Scholar 

  18. Suh C, Kang YK, Roh JL, et al. Prognostic value of tumor 18F-FDG uptake in patients with untreated extranodal natural killer/T-cell lymphomas of the head and neck. J Nucl Med 2008;49(11):1783-9.

    Article  PubMed  Google Scholar 

  19. Bishu S, Quigley JM, Schmitz J, et al. F-18-fluoro-deoxy-glucose positron emission tomography in the assessment of peripheral T-cell lymphomas. Leuk Lymphoma 2007;48(8):1531-8.

    Article  PubMed  CAS  Google Scholar 

  20. Horwitz SM. Management of peripheral T-cell non-Hodgkin’s lymphoma. Curr Opin Oncol 2007;19(5):438-43.

    Article  PubMed  Google Scholar 

  21. Pfreundschuh M, Trumper L, Kloess M, et al. Two-weekly or 3-weekly CHOP chemotherapy with or without etoposide for the treatment of young patients with good-prognosis (normal LDH) aggressive lymphomas: results of the NHL-B1 trial of the DSHNHL. Blood 2004;104(3):626-33.

    Article  PubMed  CAS  Google Scholar 

  22. Aviles A, Castaneda C, Neri N, et al. Results of a phase III clinical trial: CHOP versus CMED in peripheral T-cell lymphoma unspecified. Med Oncol 2008;25(3):360-4.

    Article  PubMed  CAS  Google Scholar 

  23. Escalon MP, Liu NS, Yang Y, et al. Prognostic factors and treatment of patients with T-cell non-Hodgkin lymphoma: the M. D. Anderson Cancer Center experience. Cancer 2005;103(10):2091-8.

    Article  PubMed  Google Scholar 

  24. Dearden C. The role of alemtuzumab in the management of T-cell malignancies. Semin Oncol 2006;33(2 Suppl 5):S44-52.

    Article  Google Scholar 

  25. O’Leary HM, Savage KJ. Novel therapies in peripheral T-cell lymphomas. Curr Oncol Rep 2008;10(5):404-11.

    Article  PubMed  Google Scholar 

  26. Ravandi F, Kantarjian H, Jones D, Dearden C, Keating M, O’Brien S. Mature T-cell leukemias. Cancer 2005;104(9):1808-18.

    Article  PubMed  CAS  Google Scholar 

  27. Mourad N, Mounier N, Briere J, et al. Clinical, biologic, and pathologic features in 157 patients with angioimmunoblastic T-cell lymphoma treated within the Groupe d’Etude des Lymphomes de l’Adulte (GELA) trials. Blood 2008;111(9):4463-70.

    Article  PubMed  CAS  Google Scholar 

  28. Lee J, Suh C, Park YH, et al. Extranodal natural killer T-cell lymphoma, nasal-type: a prognostic model from a retrospective multicenter study. J Clin Oncol 2006;24(4):612-8.

    Article  PubMed  Google Scholar 

  29. Gallamini A, Stelitano C, Calvi R, et al. Peripheral T-cell lymphoma unspecified (PTCL-U): a new prognostic model from a retrospective multicentric clinical study. Blood 2004;103(7):2474-9.

    Article  PubMed  CAS  Google Scholar 

  30. Went P, Agostinelli C, Gallamini A, et al. Marker expression in peripheral T-cell lymphoma: a proposed clinical-pathologic prognostic score. J Clin Oncol 2006;24(16):2472-9.

    Article  PubMed  CAS  Google Scholar 

  31. Cheson BD. New staging and response criteria for non-Hodgkin lymphoma and Hodgkin lymphoma. Radiol Clin North Am 2008;46(2):213-23. vii.

    Article  PubMed  Google Scholar 

  32. Morris JC, Waldmann TA, Janik JE. Receptor-directed therapy of T-cell leukemias and lymphomas. J Immunotoxicol 2008;5(2):235-48.

    Article  PubMed  CAS  Google Scholar 

  33. Chen AI, Advani RH. Beyond the guidelines in the treatment of peripheral T-cell lymphoma: new drug development. J Natl Compr Canc Netw 2008;6(4):428-35.

    PubMed  CAS  Google Scholar 

  34. Shustov AR, Savage KJ. Does high-dose therapy and autologous hematopoietic stem cell transplantation have a role in the primary treatment of peripheral T-cell lymphomas? Hematology Am Soc Hematol Educ Program 2008;2008:39-41.

    Google Scholar 

  35. Gutierrez A, Caballero MD, Perez-Manga G, Rodriguez J. Hematopoietic SCT for peripheral T-cell lymphoma. Bone Marrow Transplant 2008;42(12):773-81.

    Article  PubMed  CAS  Google Scholar 

  36. Paolo C, Lucia F, Anna D. Hematopoietic stem cell transplantation in peripheral T-cell lymphomas. Leuk Lymphoma 2007;48(8):1496-501.

    Article  PubMed  CAS  Google Scholar 

  37. Kim MK, Kim S, Lee SS, et al. High-dose chemotherapy and autologous stem cell transplantation for peripheral T-cell lymphoma: complete response at transplant predicts survival. Ann Hematol 2007;86(6):435-42.

    Article  PubMed  CAS  Google Scholar 

  38. Reimer P, Rudiger T, Geissinger E, et al. Autologous stem-cell transplantation as first-line therapy in peripheral T-cell lymphomas: results of a prospective multicenter study. J Clin Oncol 2009;27(1):106-13.

    Article  PubMed  CAS  Google Scholar 

  39. Yang DH, Kim WS, Kim SJ, et al. 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 Transplant 2009;15(1):118-25.

    Article  PubMed  CAS  Google Scholar 

  40. Aljurf M, Zaidi SZ. Chemotherapy and hematopoietic stem cell transplantation for adult T-cell lymphoblastic lymphoma: current status and controversies. Biol Blood Marrow Transplant 2005;11(10):739-54.

    Article  PubMed  CAS  Google Scholar 

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Herling, M. (2010). Clinical Management of Non-cutaneous T-cell and NK-cell Malignancies. In: Jones, D. (eds) Neoplastic Hematopathology. Contemporary Hematology. Humana Press, Totowa, NJ. https://doi.org/10.1007/978-1-60761-384-8_23

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  • DOI: https://doi.org/10.1007/978-1-60761-384-8_23

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  • Publisher Name: Humana Press, Totowa, NJ

  • Print ISBN: 978-1-60761-383-1

  • Online ISBN: 978-1-60761-384-8

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