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Intestinal T-cell lymphoma

Written2008-07Antonio Cuneo, Francesco Cavazzini, Gian Matteo Rigolin
Hematology Section, Dept. Of Biomedical Sciences, University of Ferrara, 44100 Ferrara Italy

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Identity

ICD-Topo C420,C421,C424 BLOOD, BONE MARROW, & HEMATOPOIETIC SYS
ICD-Morpho 9717/3 Enteropathy-associated T-cell lymphoma
Atlas_Id 2101
Other namesEnteropathy-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

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
 
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
 

Citation

This paper should be referenced as such :
Cuneo, A ; Cavazzini, F ; Rigolin, GM
Intestinal T-cell lymphoma
Atlas Genet Cytogenet Oncol Haematol. 2009;13(7):512-512.
Free journal version : [ pdf ]   [ DOI ]
On line version : http://AtlasGeneticsOncology.org/Anomalies/IntestinalTlymphoID2101.html


Other genes implicated (Data extracted from papers in the Atlas) [ 1 ]

Genes IDH2

External links

COSMICHisto = - Site = haematopoietic_and_lymphoid_tissue (COSMIC)
arrayMapTopo ( C42) Morph ( 9717/3) - arrayMap (UZH-SIB Zurich)  [auto + random 100 samples .. if exist ]   [tabulated segments]
 
 
Disease databaseIntestinal T-cell lymphoma
REVIEW articlesautomatic search in PubMed
Last year articlesautomatic search in PubMed
All articlesautomatic search in PubMed


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