Table 7 Comparison of different types of T and NK cell lymphoproliferative disorders and lymphomas involving the gastrointestinal tract (GIT).
Indolent T-cell lymphoma of the GIT | Indolent NK-cell LPD of the GIT | Enteropathy-associated T-cell lymphoma | Monomorphic epitheliotropic intestinal T-cell lymphoma | Extranodal NK/T-cell lymphoma | |
|---|---|---|---|---|---|
Major clinical presentation | Abdominal symptoms | Asymptomatic or nonspecific GI symptoms | Abdominal symptoms; bowel perforation or obstruction common. | Abdominal symptoms; bowel perforation or obstruction common. | Abdominal symptoms; bowel perforation common. |
Association with celiac disease | – | – | + | – | – |
Clinical course | Chronic persistent or relapsing | Usually spontaneous regression, but may persist or develop new lesions | Aggressive | Aggressive | Aggressive |
Commonest localization in GIT | Small bowel or colon | Stomach, small and large intestines | Small intestine | Small intestine | Small and large intestines |
Depth of involvement | Superficial | Superficial | Deep | Deep | Deep |
Cytomorphology | Small lymphoid cells with minimal nuclear atypia | Atypical medium-sized cells with pale cytoplasm and eosinophilic granules | Pleomorphic large or medium-sized cells, often with prominent inflammatory background | Monomorphic small to medium-sized cells | Variable cytomorphology, from small to medium-sized to large cells |
Epitheliotropism | −/ focal | −/ minimal | + | + | – |
Necrosis | – | – | +/− | Usually – | + |
EBV association | – | – | – | – | + |
Lineage | T cell, CD4+ >CD8+ | NK cell | T cell, most often CD4-, CD8- | T cell, most often CD8+ | NK cell (commoner) or T cell |
Molecular genetics | JAK2::STAT3 fusion; mutations of JAK-STAT pathway genes and epigenetic modifier genes | JAK3 mutation | Gains of 9q34; loss of 16q12; mutations of JAK-STAT pathway genes (commonly JAK1, STAT3) | Gains of 9q34; loss of 16q12; mutations of SETD2 and JAK-STAT pathway genes (commonly JAK3, STAT5B) | 6q21-25 deletion; Mutations of JAK-STAT pathway genes, epigenetic regulators, tumor suppressor genes (TP53, MGA) and RNA helicase (DDX3X) |
