| Clinical features | Histology | Immunophenotype | Outcome |
---|---|---|---|---|
Indolent digestive T-cell lymphoproliferative disease | Diarrhoea, abdominal pain | - Crypt hyperplasia, variable degrees of villous atrophy - Non-destructive superficial infiltrate of small uniform T-cells mostly lamina propria-based - Infiltration into submucosa observed in some cases - No evident major epitheliotropism | CD3+, CD8+ or CD4+, CD2+, CD5+/−, CD7+/−, CD30-, CD56-, TCRαβ+ | Indolent chronic relapsing course |
Enteropathy associated T-cell lymphoma (EATL) | Overt or silent gluten-sensitive enteropathy | - Crypt hyperplasia, villous atrophy - Pleomorphic medium- to large-sized neoplastic lymphocytes with transmural infiltration - Presence of other mixed inflammatory cells such as histiocytes and eosinophils - Intraepithelial lymphocytosis present in non-tumoral mucosa and in epithelium distant from the main mass | CD3+, CD5-, CD8−/+, CD56-, CD103+, often CD30+, cytotoxic phenotype +/−, TCR αβ + (usually) | Aggressive |
Monomorphic epitheliotropic T-cell lymphoma | Occurs without a history of coeliac disease | - No crypt hyperplasia, possible villous atrophy - Monomorphic infiltrate with epitheliotropism - Transmural infiltration - No associated inflammatory background | CD3+, CD5-, CD4-, CD8+, CD56+, cytotoxic phenotype, CD30-, TCR γδ + (usually) | Aggressive |