Peripheral T-cell lymphoma not otherwise specified explained

Peripheral T-cell lymphoma-Not-Otherwise-Specified

Peripheral T-cell lymphoma not otherwise specified (PTCL-NOS), is a subtype of peripheral T-cell lymphoma. Peripheral T-cell lymphoma (PTCL) is defined as a diverse group of aggressive lymphomas that develop from mature-stage white blood cells called T-cells and natural killer cells (NK cells) (see figure for an overview of PTCL subtypes). PTCL is a type of non-Hodgkin's lymphoma (NHL).[1] PTCL specifically affects T-cells rather than B-cells, and results when T-cells develop and grow abnormally.

About 30% of PTCL-NOS cases exhibit malignant T cells that are infected with the Epstein-Barr virus (EBV). When associated with EBV, PTCL-NOS is classified as one of the Epstein-Barr virus-associated lymphoproliferative diseases (see Epstein-Barr virus-associated peripheral T cell lymphoma, not otherwise specified) but the relationship of EBV to the development and progression of Epstein-Barr virus-associated PTCL-NOS is unclear.[2]

PTCL-NOS, the most common subtype of PTCL, is aggressive and predominantly nodal. There are two morphologic variants: the T-zone lymphoma variant and the lymphoepithelioid cell variant.[3] [4]

Treatment

Currently PTCL is treated similarly to B-cell lymphomas. However, in recent years, scientists have developed techniques to better recognize the different types of lymphomas, such as PTCL. It is now understood that PTCL behaves differently from B-cell lymphomas and therapies are being developed that specifically target these types of lymphoma. Currently, however, there are no therapies approved by the US Food and Drug Administration (FDA) specifically for PTCL. Anthracycline-containing chemotherapy regimens are commonly offered as the initial therapy. Some patients may receive a stem cell transplant.[7] [8] [9] [10] [11] [12] [13] [14] Novel approaches to the treatment of PTCL in the relapsed or refractory setting are under investigation.

Pralatrexate and cerdulatinib are some of the compounds currently under investigations for the treatment of PTC.

Notes and References

  1. Swerdlow SH, WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues, 2008
  2. Rezk SA, Zhao X, Weiss LM . Epstein - Barr virus - associated lymphoid proliferations, a 2018 update . Human Pathology . 79. 18–41. June 2018 . 29885408 . 10.1016/j.humpath.2018.05.020 . 47010934 .
  3. Vose JM . Peripheral T-cell non-Hodgkin's lymphoma . Hematol. Oncol. Clin. North Am. . 22 . 5 . 997–1005, x . October 2008 . 18954748 . 10.1016/j.hoc.2008.07.010 .
  4. O’Connor, Owen. Getting the Facts; Peripheral T-Cell Lymphoma. [electronic version] Retrieved May 19, 2009, from http://www.lymphoma.org/atf/cf/%7B0363CDD6-51B5-427B-BE48-E6AF871ACEC9%7D/PTCL.PDF
  5. Stein H, Bonk A, Tolksdorf G, Lennert K, Rodt H, Gerdes J . Immunohistologic analysis of the organization of normal lymphoid tissue and non-Hodgkin's lymphomas . J. Histochem. Cytochem. . 28 . 8 . 746–60 . August 1980 . 7003001 . 10.1177/28.8.7003001 . free .
  6. Daneshbod Y . Cytologic findings of peripheral T-cell lymphoma (PTCL) with high epitheloid cell content (Lennert's lymphoma) in imprint smear. A case report . CytoJournal . 3 . 3 . 2006 . 16460569 . 1434763 . 10.1186/1742-6413-3-3 . free .
  7. Reimer P, Rüdiger T, Geissinger E, etal . Autologous stem-cell transplantation as first-line therapy in peripheral T-cell lymphomas: results of a prospective multicenter study . J. Clin. Oncol. . 27 . 1 . 106–13 . January 2009 . 19029417 . 10.1200/JCO.2008.17.4870 . July 6, 2011 . https://archive.today/20120803120417/http://www.jco.org/cgi/pmidlookup?view=long&pmid=19029417 . August 3, 2012 . dead .
  8. Mercadal S, Briones J, Xicoy B, etal . Intensive chemotherapy (high-dose CHOP/ESHAP regimen) followed by autologous stem-cell transplantation in previously untreated patients with peripheral T-cell lymphoma . Ann. Oncol. . 19 . 5 . 958–63 . May 2008 . 18303032 . 10.1093/annonc/mdn022 . free .
  9. Rodríguez J, Conde E, Gutiérrez A, etal . Frontline autologous stem cell transplantation in high-risk peripheral T-cell lymphoma: a prospective study from The Gel-Tamo Study Group . Eur. J. Haematol. . 79 . 1 . 32–8 . July 2007 . 17598836 . 10.1111/j.1600-0609.2007.00856.x . 32509254 .
  10. Corradini P, Tarella C, Zallio F, etal . Long-term follow-up of patients with peripheral T-cell lymphomas treated up-front with high-dose chemotherapy followed by autologous stem cell transplantation . Leukemia . 20 . 9 . 1533–8 . September 2006 . 16871285 . 10.1038/sj.leu.2404306 . 22959229 .
  11. d’Amore F, et al. Blood. 2006;108:A401
  12. Gisselbrecht C, Lepage E, Molina T, etal . Shortened first-line high-dose chemotherapy for patients with poor-prognosis aggressive lymphoma . J. Clin. Oncol. . 20 . 10 . 2472–9 . May 2002 . 12011124 . https://archive.today/20120903220131/http://www.jco.org/cgi/pmidlookup?view=long&pmid=12011124 . dead . 2012-09-03 . 10.1200/JCO.2002.02.125 .
  13. Deconinck E, Lamy T, Foussard C, etal . Autologous stem cell transplantation for anaplastic large-cell lymphomas: results of a prospective trial . Br. J. Haematol. . 109 . 4 . 736–42 . June 2000 . 10929023 . 10.1046/j.1365-2141.2000.02098.x. 38197607 .
  14. Haioun C, Lepage E, Gisselbrecht C, etal . Survival benefit of high-dose therapy in poor-risk aggressive non-Hodgkin's lymphoma: final analysis of the prospective LNH87-2 protocol—a groupe d'Etude des lymphomes de l'Adulte study . J. Clin. Oncol. . 18 . 16 . 3025–30 . August 2000 . 10944137 . https://archive.today/20120906152354/http://www.jco.org/cgi/pmidlookup?view=long&pmid=10944137 . dead . 2012-09-06 . 10.1200/JCO.2000.18.16.3025 .

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