Intestinal T-cell lymphoma

2008-07-01   Francesco Cavazzini , Gian Matteo Rigolin , Antonio Cuneo 

1.Hematology Section, Dept. Of Biomedical Sciences, University of Ferrara, 44100 Ferrara Italy

Clinics and Pathology

Phenotype stem cell 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).

Bibliography

Pubmed IDLast YearTitleAuthors
145639522003High frequency of genetic aberrations in enteropathy-type T-cell lymphoma.Baumgärtner AK et al
128609532003Intestinal non-Hodgkin's lymphoma: a multicenter prospective clinical study from the German Study Group on Intestinal non-Hodgkin's Lymphoma.Daum S et al
147435092004Loss of heterozygosity at chromosome 9p21 is a frequent finding in enteropathy-type T-cell lymphoma.Obermann EC et al
124145112002Chromosomal gains at 9q characterize enteropathy-type T-cell lymphoma.Zettl A et al

Citation

Francesco Cavazzini ; Gian Matteo Rigolin ; Antonio Cuneo

Intestinal T-cell lymphoma

Atlas Genet Cytogenet Oncol Haematol. 2008-07-01

Online version: http://atlasgeneticsoncology.org/haematological/2101/intestinal-t-cell-lymphoma

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