Peripheral T-cell lymphoma not otherwise specified

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

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.

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]
  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.