Table 1.
Lymphoproliferative disorders of T/NK-cell origin with GIT involvement.
| Variation | ENKTL-NT | CAEBV | EATL | MEITL | ITLPD |
|---|---|---|---|---|---|
| Sites of GIT (in order of frequency) |
Small bowel Large bowel Stomach Esophagus |
Large bowel Small bowel Stomach |
Small bowel; Large bowel Stomach |
Small bowel; large bowel; stomach | All sites of GIT; Small bowel or large bowel (more often) |
| Macroscopic features | Mucosal ulcer or ulcerating mass | Ulcer | Ulcerating lesion or stricture | Tumor mass | Subtle features: hyperaemic mucosa; nodularity; Prominent folds; polyps |
| Coeliac disease | No | No | Yes | No | No |
| Histology | Cells of variable size; Admixed inflammation; Necrosis; Angioinvasive/angiocentric pattern |
Infiltrate within mucosa, rarely submucosa or muscle layer involvement; Small/medium-sized cells often with mild atypia |
Medium/ large- sized cells; Admixed inflammation; Necrosis; Angioinvasive/ angiocentric pattern |
Monomorphic Medium- Sized cells; Epitheliotropism (often) |
Non-destructive mucosal infiltrate (rarely muscolaris mucosae or submucosa involved) |
| Cell of origin | NK-cell often; T-cell |
T-cell (59%); NK-cell (41%); B-cell rarely | T-cell | T-cell | T-cell |
| IHC | NK cell origin: CD2+ cCD3+ CD56- cytotoxic molecules+ CD7+/− CD30+/− sCD3−, CD4− CD8− CD5− CD16− CD57− MATK+. T-cell origin: sCD3+ CD5+ CD8+ MATK+ |
T-cell origin: CD4 > CD8 |
CD3+ CD7+ CD103+ CD5− CD4− CD8− often cytotoxic molecules+ CD30+ (often) TCRB+ (in some cases) TCRG+ (in some cases) |
CD3+ CD8+ CD56+ CD5− TCRG+ (often) TIA1+ CD20 aberrant+ (20% of cases) MATK+ |
CD3+ CD8+ often; CD4+ some cases; CD4− CD8− rarely; CD2+ CD5+ CD7+/− TIA1+ Granzyme B− TCR alfa beta+ CD56- |
| EBER-ISH | Positive | Positive | Negative | Negative | Negative |
| Proliferative fraction | High | Low | High | High | Low |
| T-cell clonality | T-cell clonality in cases of T-cell origin | T-cell clonality; more rarely oligoclonality or polyclonality |
T-cell clonality | T-cell clonality | |
| Clinical course | Aggressive | Variable (NK forms: more favorable course) | Aggressive | Aggressive | Indolent |
CAEBV: chronic active Epstein–Barr virus infection; EBER-ISH: EBV-encoded RNA in situ hybridization; EATL: enteropathy-associated T-cell lymphoma; ENKTL-NT: extranodal NK/T-cell lymphoma, nasal type; gi-ITLPD: indolent T-cell lymphoproliferative disorder of the gastrointestinal tract; GIT: gastrointestinal tract; IHC: immunohistochemistry; MATK: megakaryocyte-associated tyrosine kinase; MEITL: monomorphic epitheliotropic intestinal T-cell lymphoma; TCRB: T-cell receptor beta gene rearrangement; TCRG: T-cell receptor gamma gene rearrangement.