Enteropathy-associated T-cell lymphoma classification

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Classification

  • Type I enteropathy-associated T-cell lymphoma
  • Type II enteropathy-associated T-cell lymphoma
Enteropathy-associated T-cell lymphoma classification[1][3][4]
Name Description
Classical enteropathy-associated T-cell lymphoma (Type I Enteropathy-associated T-cell lymphoma)
  • Covers 80-90% of all enteropathy-associated T-cell lymphoma cases.
  • Is usually associated with refractory celiac disease that is sometimes accompanied by intestinal ulceration (ulcerative jejunitis)
  • Most show adult onset disease or are diagnosed as having celiac disease in the same clinical episode in which the lymphoma is diagnosed.
  • Frequently has large-cell or pleomorphic cytology.
  • Seldom expresses CD8 and CD56.
Monomorphic enteropathy-associated T-cell lymphoma (Type II enteropathy-associated T-cell lymphoma)
  • Encountered in Asia and other regions where celiac disease is rare.
  • Is sporadic, seldom associated with celiac disease.
  • Covers 10-20% of all enteropathy-associated T-cell lymphoma cases.
  • Is characterized by monomorphic cytology.
  • Frequent expression of CD8 and CD56.
  • The tumor forms an ulcerating mucosal mass that invades the wall of the intestine.
  • Poor prognosis, death occurs from abdominal complications such as small bowel obstruction, perforation.
  1. ↑ 1.0 1.1 Enteropathy-associated T-cell lymphoma. BioMed Central. http://diagnosticpathology.biomedcentral.com/articles/10.1186/1746-1596-7-172. Accessed on January 28, 2016
  2. ↑ V G, Kudva R, Amprayil AJ (October 2014). "Enteropathy associated T cell lymphoma - a case report of an uncommon extranodal T cell lymphoma". J Clin Diagn Res. 8 (10): FD10–2. doi:10.7860/JCDR/2014/9740.4999. PMC 4253173. PMID 25478355.
  3. ↑ Enteropathy-associated T-cell lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd5315/. Accessed on January 26, 2016
  4. ↑ Hussain N, Hussain F, Chatterjee T, Upalakalin JN, Lynch T (2018). "An unexpected deterrent in diagnosing refractory celiac disease and enteropathy-associated T-cell lymphoma: a gluten-free diet". J Community Hosp Intern Med Perspect. 8 (4): 233–236. doi:10.1080/20009666.2018.1483693. PMC 6116147. PMID 30181834.
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