Enteropathy-associated T-cell lymphoma natural history, complications and prognosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Nima Nasiri, M.D.[2]

Overview

Enteropathy-associated T-cell lymphoma is usually a fast-growing (aggressive) lymphoma. Common complications of enteropathy-associated T-cell lymphoma include ulcer, obstruction, and perforation of small intestine. Prognosis is generally poor.

Natural History

  • Enteropathy-associated T-cell lymphoma is usually a fast-growing (aggressive) lymphoma.[1]
  • It is associated with celiac disease (sprue).
  • Most adults are diagnosed with celiac disease at the same time as their lymphoma or shortly before their lymphoma is diagnosed.
  • Enteropathy-associated T-cell lymphoma may spread to the liver, spleen, lymph nodes, gallbladder, stomach, colon or skin.

Complications

  • Common complications of enteropathy-associated T-cell lymphoma include:[1][2]

Prognosis

  • The prognosis for people with enteropathy-associated T-cell lymphoma is often not very good.[1][3]
  • Recurrences most frequent in the small intestine.[4]
  • The most influential prognostic factor is bulky nature of disease, defined by a tumor mass greater than 5 cm. [5] [6]
  • Autologous stem cell transplantation is a useful way of treatment for selected patients with enteropathy-associated T-cell lymphoma and can control disease progress.

References

  1. 1.0 1.1 1.2 Enteropathy-associated T-cell lymphoma . Canadian Cancer Society. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/enteropathy-associated-t-cell-lymphoma/?region=on Accessed on January 27, 2016
  2. Kabul S, Uğraş N, Yerci Ö, Öztürk E (2018). "Perforation of the small intestine caused by enteropathy-associated T cell lymphoma". Turk J Surg. 34 (3): 253–255. doi:10.5152/turkjsurg.2017.3278. PMC 6173604. PMID 30302434.
  3. Nijeboer P, de Baaij LR, Visser O, Witte BI, Cillessen SA, Mulder CJ, Bouma G (June 2015). "Treatment response in enteropathy associated T-cell lymphoma; survival in a large multicenter cohort". Am. J. Hematol. 90 (6): 493–8. doi:10.1002/ajh.23992. PMID 25716069.
  4. Enteropathy-associated T-cell lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd5315/. Accessed on January 27, 2016
  5. Delabie J, et al. (July 2011). "Enteropathy-associated T-cell lymphoma: clinical and histological findings from the International Peripheral T-Cell Lymphoma Project". Blood. 118 (148): 148. doi:10.1182/blood-2011-02-335216.
  6. Kumar, A.; Burger, I. A.; Zhang, Z.; Drill, E. N.; Migliacci, J. C.; Ng, A.; LaCasce, A.; Wall, D.; Witzig, T. E.; Ristow, K.; Yahalom, J.; Moskowitz, C. H.; Zelenetz, A. D. (2016). "Definition of bulky disease in early stage Hodgkin lymphoma in computed tomography era: prognostic significance of measurements in the coronal and transverse planes". Haematologica. 101 (10): 1237–1243. doi:10.3324/haematol.2016.141846. ISSN 0390-6078.


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