9717/3 Intestinal T-cell lymphoma


Synonyms

Enteropathy type intestinal T-cell lymphoma
Enteropathy associated T-cell lymphoma

Definitions

Tumours of haematopoietic and lymphoid tissues
ICD-O-3 topography code: C42 and C77
ICD10: C86.2
           

/span>nteropathy-associated T-cell lymphoma (EATL) is an intestinal tumour of intra?epithelial T lymphocytes, showing varying degrees of transformation but usually presenting as a tumour composed of large lymphoid cells, often with an inflammatory background. The adjacent small intestinal mucosa shows villous atrophy with crypt hyperplasia. In 10−20% of cases, the lymphoma is composed of monomorphic medium-sized cells. This monomorphic variant (type II EATL) may occur sporadically, without risk factors for coeliac disease (Table 11.03).


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Small intestine
ICD-O-3 topography code: C17
ICD10: C86.2
           

Enteropathy-associated intestinal T-cell lymphoma (EATL) is an intestinal tumour of intraepithelial T-lymphocytes composed of large, often polymorphic lymphoid cells, which are negative for CD56. It is also referred to as Type I EATL. The disease is uncommon in most parts of the world, but is seen with increasing frequency in areas with a high prevalence of coeliac disease, e.g. Northern Europe. Most patients with EATL show adult-onset disease or are diagnosed as having coeliac disease in the same clinical episode in which the lymphoma is diagnosed. Patients present with abdominal pain, most often as emergencies associated with intestinal perforation and/or obstruction. Histologically, the degree of infiltration is highly variable. Prognosis is usually poor, with death frequently resulting from abdominal complications in patients already weakened by uncontrolled malabsorption, but long-term survivals are recorded
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Bosman FT, Carneiro F, Hruban RH, Theise ND (Eds.)
WHO Classification of Tumours of the Digestive System.
4th Edition
International Agency for Research on Cancer: Lyon 2010



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