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: Sowminya Arikapudi, M.B,B.S. [2]

Overview

Common complications of enteropathy-associated T-cell lymphoma include ulcer, obstruction, and perforation of small intestine.[1]

Natural History[1]

  • Enteropathy-associated T-cell lymphoma is associated with celiac disease (sprue)
  • People with celiac disease cannot tolerate gluten, a protein found in many grains, such as wheat, rye and barley (gluten sensitivity)
  • A gluten-free diet helps prevent enteropathy-associated T-cell lymphoma from developing, so this type of lymphoma does not commonly occur in people diagnosed with celiac disease at a young age
  • Most adults are diagnosed with celiac disease at the same time as their lymphoma or shortly before their lymphoma is diagnosed

Complications

  • Common complications of enteropathy-associated T-cell lymphoma include:[1]
  • Ulcer in small intestine
  • Obstruction of small intestine
  • Perforation of small intestine

Prognosis

  • EATL is usually a fast-growing (aggressive) lymphoma. The prognosis for people with EATL is often not very good.[1]
  • Recurrences most frequent in the small intestine[2]
  • According to the Peripheral T-Cell Lymphoma Project, median overall survival is ten months, while median failure-free survival is only six months
  • The peripheral index for T-cell lymphoma is useful in defining prognosis for enteropathy-associated T-cell lymphoma
  • Among the most influential prognostic factors is bulky disease, defined by a tumor mass greater than 5 cm[3]
  • Autologous stem cell transplantation is feasible for selected patients with enteropathy-associated T-cell lymphoma and can yield durable disease control in a significant proportion of these patients
  • One study found a trend for better survival in patients transplanted in first complete or partial remission at four years (66% vs. 36%; P = .062)[4]

References

  1. 1.0 1.1 1.2 1.3 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. Enteropathy-associated T-cell lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd5315/. Accessed on January 27, 2016
  3. 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.
  4. Jantunen, E; Boumendil, A; Finel, H; Luan, J. J.; Johnson, P; Rambaldi, A; Haynes, A; Duchosal, M. A.; Bethge, W; Biron, P; Carlson, K; Craddock, C; Rudin, C; Finke, J; Salles, G; Kroschinsky, F; Sureda, A; Dreger, P; Lymphoma Working Party of the EBMT (2013). "Autologous stem cell transplantation for enteropathy-associated T-cell lymphoma: A retrospective study by the EBMT". Blood. 121 (13): 2529–32. doi:10.1182/blood-2012-11-466839. PMID 23361910.


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