Intestinal T-cell lymphoma

Identity

Other names

Enteropathy-type T-cell lymphoma

Clinics and Pathology

Phenotype / cell stem origin	The disease originates from a CD3+, CD7+ T-lymphocyte lacking CD4 and CD8 expression.
Epidemiology	The disease affects mainly the adult, with male predominance, and it is frequently associated with gluten-sensitive entheropathy.
Pathology	The disease consists of ulcerated lesions involving the small intestine. Perforation may occur. Small and larger atypical lymphocytes with pale cytoplasm infiltrate the epithelial mucosa of the villi. The TCR-Beta and TCR-Gamma genes are clonally rearranged.
Treatment	Multiagent chemotherapy (CHOP or CHOP-like regimes) was used.
Evolution	The disease may derive from patients with coeliac disease not responding to gluten-free diet. The lymphoma may spread to regional lymph nodes.
Prognosis	Response to chemotherapy is suboptimal and patients are vulnerable to toxicity of treatment due to intestinal symptoms and malnutrition preceding the diagnosis of lymphoma. Survival at 2 years was 28% in a study (Daum et al., 2003).

Cytogenetics

Cytogenetics Molecular

Extra copies of chromosome 9q centered around the 9q33-34 region was detected by CGH and FISH studies (Zettl et al., 2002). 9p deletion with p16 loss was found in 18% of the cases, and loss of heterozygosity at 9p21 with loss of p16 expression was documented in approximately half of those cases with a large cell component (Obermann et al., 2004). DNA gains may involve the 5q33-34 and 7q31 regions, with a 30% frequency. Loss of chromosome material was detected at 6p24; 7p21, 17q23-25 and 17p13 (Baumgartner et al., 2003).

Bibliography

Chromosomal gains at 9q characterize enteropathy-type T-cell lymphoma.

Zettl A, Ott G, Makulik A, Katzenberger T, Starostik P, Eichler T, Puppe B, Bentz M, Muller-Hermelink HK, Chott A.

Am J Pathol. 2002; 161: 1635-1645.

PMID 12414511

High frequency of genetic aberrations in enteropathy-type T-cell lymphoma.

Baumgartner AK, Zettl A, Chott A, Ott G, Muller-Hermelink HK, Starostik P.

Lab Invest. 2003; 83: 1509-1516.

PMID 14563952

Intestinal non-Hodgkin's lymphoma: a multicenter prospective clinical study from the German Study Group on Intestinal non-Hodgkin's Lymphoma.

Daum S, Ullrich R, Heise W, Dederke B, Foss HD, Stein H, Thiel E, Zeitz M, Riecken EO.

J Clin Oncol. 2003; 21: 2740-2746.

PMID 12860953

Loss of heterozygosity at chromosome 9p21 is a frequent finding in enteropathy-type T-cell lymphoma.

Obermann EC, Diss TC, Hamoudi RA, Munson P, Wilkins BS, Camozzi ML, Isaacson PG, Du MQ, Dogan A.

J Pathol. 2004; 202: 252-62.

PMID 14743509

Contributor(s)

Written	07-2008	Antonio Cuneo, Francesco Cavazzini, Gian Matteo Rigolin
		Hematology Section, Dept. Of Biomedical Sciences, University of Ferrara, 44100 Ferrara Italy

Citation

This paper should be referenced as such :

Cuneo A, Cavazzini F, Rigolin GM . Intestinal T-cell lymphoma. Atlas Genet Cytogenet Oncol Haematol. July 2008 . URL : http://AtlasGeneticsOncology.org/Genes/IntestinalTlymphoID2101.html

© Atlas of Genetics and Cytogenetics in Oncology and Haematology

indexed on : Sat Dec 6 18:01:27 2008

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