Enteropathy-Associated T-cell lymphoma

Enteropathy-associated T-cell lymphoma
Classification and external resources
ICD-O: 9717/3

Enteropathy-Associated T-cell Lymphoma (EATL), also enteropathy-type T-cell lymphoma (ETTL), is a type of T-cell lymphoma that affects the small intestine. It is the most common primary gastrointestinal T-cell lymphoma, arising from the T cells that are found between the cells that line the small intestinal (brush border cells or small intestinal epithelial cells).[1] These cancerous T-cells are a possible consequence of refractory cases of coeliac disease or in chronic, untreated cases in genetically susceptible individuals.

Epidemiology

EATL is most frequent in Europe, where it represents 9.4% of all peripheral T cell lymphomas. Association with celiac disease is consistently demonstrated in only 30% of patients. The global incidence of this lymphoma is rare, being about 0.5 to 1 per million.[2]

Classification

EATL can be classified as an extranodal peripheral T Cell lymphoma, category it shares with Hepatosplenic T cell lymphoma, and Panniculitic T Cell lymphoma. It can be further classified in type I and II EATL.[2]

Genetics

Enteropathy associated T-cell lymphoma (EATL) is environmentally induced as a result of the consumption of Refractory coeliac disease is no longer favorably responsive to wheat-gluten abstinence. Beyond the RCD1 stage, many drugs are not effective, and undetected coeliac disease leading to de novo EATL generally has a poor outcome.

The genetic association with celiac disease and HLA loci defines type I EATL. Type II doesn´t show these associations and frequently presents with bulky disease.

Early recognition of coeliac disease, particularly with a focus on DQ2 homozygotes and in affected family members, is the only effective prevention, though bone marrow transplant was suggested as a treatment during early RCD2.[8]

Staging

Bone marrow involvement is rare in this disease.

Treatment

EATL has mainly been treated with CHOP and CHOP-like regimens; exposure to anthracycline drugs has been recognized as a favorable prognostic factor.

Prognosis

According to the Peripheral T cell lymphoma project, median overall survival is 10 months, while median failure free survival is only 6 months. The international prognostic index is not useful in defining prognosis in this entity, but Peripheral Index for T cell lymphoma is. Among the most influential prognostic factors is bulky disease, defined by a tumor mass >5cm.[2]

See also

References

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