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Peripheral T-cell lymphoma not otherwise specified

(Redirected from 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).[2] PTCL specifically affects T-cells rather than B-cells, and results when T-cells develop and grow abnormally.

Peripheral T-cell lymphoma-Not-Otherwise-Specified
SpecialtyOncology Edit this on Wikidata
Subtypes of T-cell lymphoma[1]

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.[3]

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.[4][5]

  • T-zone lymphoma is so named for its involvement in a specific area of the lymph node that consists of a dense accumulation of T-cells.[6]
  • Lympho-epithelioid lymphoma, also called Lennert's lymphoma, is rare and generally affects older individuals.[7]

Treatment

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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.[8][9][10][11][12][13][14][15] Novel approaches to the treatment of PTCL in the relapsed or refractory setting are under investigation.[citation needed]

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

References

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  1. ^ Rodriguez, J.; Gutierrez, A.; Martinez-Delgado, B.; Perez-Manga, G. (2009). "Current and future aggressive peripheral T-cell lymphoma treatment paradigms, biological features and therapeutic molecular _targets". Crit Rev Oncol Hematol. 71 (3): 181–198. doi:10.1016/j.critrevonc.2008.10.011. PMID 19056295.
  2. ^ Swerdlow SH, WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues, 2008
  3. ^ Rezk SA, Zhao X, Weiss LM (June 2018). "Epstein - Barr virus - associated lymphoid proliferations, a 2018 update". Human Pathology. 79: 18–41. doi:10.1016/j.humpath.2018.05.020. PMID 29885408. S2CID 47010934.
  4. ^ Vose JM (October 2008). "Peripheral T-cell non-Hodgkin's lymphoma". Hematol. Oncol. Clin. North Am. 22 (5): 997–1005, x. doi:10.1016/j.hoc.2008.07.010. PMID 18954748.
  5. ^ 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 Archived April 20, 2009, at the Wayback Machine
  6. ^ Stein H, Bonk A, Tolksdorf G, Lennert K, Rodt H, Gerdes J (August 1980). "Immunohistologic analysis of the organization of normal lymphoid tissue and non-Hodgkin's lymphomas". J. Histochem. Cytochem. 28 (8): 746–60. doi:10.1177/28.8.7003001. PMID 7003001.[permanent dead link‍]
  7. ^ Daneshbod Y (2006). "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. doi:10.1186/1742-6413-3-3. PMC 1434763. PMID 16460569.
  8. ^ Reimer P, Rüdiger T, Geissinger E, et al. (January 2009). "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. doi:10.1200/JCO.2008.17.4870. PMID 19029417. Archived from the original on August 3, 2012. Retrieved July 6, 2011.
  9. ^ Mercadal S, Briones J, Xicoy B, et al. (May 2008). "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. doi:10.1093/annonc/mdn022. PMID 18303032.
  10. ^ Rodríguez J, Conde E, Gutiérrez A, et al. (July 2007). "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. doi:10.1111/j.1600-0609.2007.00856.x. PMID 17598836. S2CID 32509254.
  11. ^ Corradini P, Tarella C, Zallio F, et al. (September 2006). "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. doi:10.1038/sj.leu.2404306. PMID 16871285. S2CID 22959229.
  12. ^ d’Amore F, et al. Blood. 2006;108:A401
  13. ^ Gisselbrecht C, Lepage E, Molina T, et al. (May 2002). "Shortened first-line high-dose chemotherapy for patients with poor-prognosis aggressive lymphoma". J. Clin. Oncol. 20 (10): 2472–9. doi:10.1200/JCO.2002.02.125. PMID 12011124. Archived from the original on September 3, 2012.
  14. ^ Deconinck E, Lamy T, Foussard C, et al. (June 2000). "Autologous stem cell transplantation for anaplastic large-cell lymphomas: results of a prospective trial". Br. J. Haematol. 109 (4): 736–42. doi:10.1046/j.1365-2141.2000.02098.x. PMID 10929023. S2CID 38197607.
  15. ^ Haioun C, Lepage E, Gisselbrecht C, et al. (August 2000). "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. doi:10.1200/JCO.2000.18.16.3025. PMID 10944137. Archived from the original on September 6, 2012.
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