The disease originates from a CD3+, CD7+ T-lymphocyte lacking CD4 and CD8 expression.

The disease affects mainly the adult, with male predominance, and it is frequently associated with gluten-sensitive entheropathy.

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.

Multiagent chemotherapy (CHOP or CHOP-like regimes) was used.

The disease may derive from patients with coeliac disease not responding to gluten-free diet. The lymphoma may spread to regional lymph nodes.

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).