T-cell LPD of the GIT Type . Clinical .... | American Society of Hematology

T-cell LPD of the GIT

Type	Clinical	Histology	Phenotype	Outcome
EATL type I	Overt or silent gluten-sensitive enteropathy	Polymorphous medium to large cells including	CD4⁻, CD8^−/+	Aggressive
		Hodgkin-like cells	TCR αβ (usually)
			or γδ
			Variable expression of CD30
EATL type II	Worldwide, not clearly linked to celiac disease	Monomorphic with epitheliotropism	TCR γδ (usually) or αβ	Aggressive
EATL type II	Worldwide, not clearly linked to celiac disease	Monomorphic with epitheliotropism	CD8⁺, CD56⁺	Aggressive
Extranodal NK-/T-cell lymphoma nasal type	Upper aerodigestive tract and small bowel most often involved	Medium to large cells	NK/cytotoxic T cells, EBV⁺	Aggressive
PTCL-NOS	No history of celiac disease	No significant epitheliotropism	Variable	Aggressive
PTCL-NOS	No history of celiac disease	No significant epitheliotropism	More often TCR silent	Aggressive
NK-cell enteropathy	Vague gastrointestinal symptoms, can involve entire GIT	Medium to large irregular cells do not invade glandular epithelium	CD56⁺	Indolent chronic relapsing course
NK-cell enteropathy	Vague gastrointestinal symptoms, can involve entire GIT		Cytotoxic granules, cytoplasmic CD3⁺	Indolent chronic relapsing course
Indolent T-cell lymphoproliferative disease of the GIT	Diarrhea, abdominal pain	Superficial infiltrate of small uniform lymphoid cells	Mostly CD8⁺ cytotoxic T cell	Indolent chronic relapsing course

Type	Clinical	Histology	Phenotype	Outcome
EATL type I	Overt or silent gluten-sensitive enteropathy	Polymorphous medium to large cells including	CD4⁻, CD8^−/+	Aggressive
		Hodgkin-like cells	TCR αβ (usually)
			or γδ
			Variable expression of CD30
EATL type II	Worldwide, not clearly linked to celiac disease	Monomorphic with epitheliotropism	TCR γδ (usually) or αβ	Aggressive
EATL type II	Worldwide, not clearly linked to celiac disease	Monomorphic with epitheliotropism	CD8⁺, CD56⁺	Aggressive
Extranodal NK-/T-cell lymphoma nasal type	Upper aerodigestive tract and small bowel most often involved	Medium to large cells	NK/cytotoxic T cells, EBV⁺	Aggressive
PTCL-NOS	No history of celiac disease	No significant epitheliotropism	Variable	Aggressive
PTCL-NOS	No history of celiac disease	No significant epitheliotropism	More often TCR silent	Aggressive
NK-cell enteropathy	Vague gastrointestinal symptoms, can involve entire GIT	Medium to large irregular cells do not invade glandular epithelium	CD56⁺	Indolent chronic relapsing course
NK-cell enteropathy	Vague gastrointestinal symptoms, can involve entire GIT		Cytotoxic granules, cytoplasmic CD3⁺	Indolent chronic relapsing course
Indolent T-cell lymphoproliferative disease of the GIT	Diarrhea, abdominal pain	Superficial infiltrate of small uniform lymphoid cells	Mostly CD8⁺ cytotoxic T cell	Indolent chronic relapsing course

EBV, Epstein-Barr virus; NOS, not otherwise specified.