Enteropathy-associated T-cell lymphoma medical therapy

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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 (EATL), also enteropathy-type T-cell lymphoma (ETTL), is a type of T-cell non-hodgkin lymphoma that affects the small intestine, it is composed of large lymphoid cells. Enteropathy-associated T-cell lymphoma has two subtypes, type I enteropathy-associated T-cell lymphoma which has a strong association with celiac disease and it is more common in western countries and type II enteropathy-associated T-cell lymphoma which is mostly found among the Asian population. Genes involved in the pathogenesis of this disease include 8q24, T-cell receptor (TCR) beta and gamma, and 16q genes. On gross pathology, multiple intestinal ulcers are characteristic findings of EATL. On microscopic histopathological analysis, monotonous cells, round or angulated vesicular nuclei, and prominent nucleoli are characteristic findings of enteropathy-associated T-cell lymphoma. There are no established causes for enteropathy-associated T-cell lymphoma. EATL must be differentiated from other diseases such as peptic ulcer, poorly-differentiated adenocarcinoma, MALT lymphoma, diffuse large B cell lymphoma, and mantle cell lymphoma.

Treatment

Medical Therapy

  • There is no treatment for enteropathy-associated T-cell lymphoma; the mainstay of therapy is supportive care.
  • The optimal therapy for enteropathy-associated T-cell lymphoma depends on the extent and the location of the lymphoma in the small intestine.[1]
  • Stem cell transplant can be helpful in those patients that are in remission phase of disease.[2]
Treatment of enteropathy-associated T-cell lymphoma[1]
Therapy Description
Supportive therapy
Surgery
Chemotherapy
  • May be offered to people who can tolerate it:
  • Following surgery
  • When the cancer cannot be removed by surgery or is extensive

References

  1. 1.0 1.1 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 26, 2016
  2. Rongey C, Micallef I, Smyrk T, Murray J (June 2006). "Successful treatment of enteropathy-associated T cell lymphoma with autologous stem cell transplant". Dig. Dis. Sci. 51 (6): 1082–6. doi:10.1007/s10620-006-8013-z. PMID 16865575.