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
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.[1]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]
- Ulcer in small intestine
- Obstruction of small intestine
- Perforation of small intestine
Prognosis
- The prognosis for people with enteropathy-associated T-cell lymphoma 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.
- The most influential prognostic factor is bulky nature of disease, defined by a tumor mass greater than 5 cm. [3] [4]
- 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).[5]
References
- ↑ 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
- ↑ Enteropathy-associated T-cell lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd5315/. Accessed on January 27, 2016
- ↑ 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.
- ↑ 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.
- ↑ 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.