Intestinal T-cell lymphoma
2008-07-01 Francesco Cavazzini , Gian Matteo Rigolin , Antonio Cuneo Affiliation1.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
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).
Genes Involved and Proteins
Article Bibliography
| Pubmed ID | Last Year | Title | Authors |
|---|---|---|---|
| 14563952 | 2003 | High frequency of genetic aberrations in enteropathy-type T-cell lymphoma. | Baumgärtner AK et al |
| 12860953 | 2003 | Intestinal non-Hodgkin's lymphoma: a multicenter prospective clinical study from the German Study Group on Intestinal non-Hodgkin's Lymphoma. | Daum S et al |
| 14743509 | 2004 | Loss of heterozygosity at chromosome 9p21 is a frequent finding in enteropathy-type T-cell lymphoma. | Obermann EC et al |
| 12414511 | 2002 | Chromosomal 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
