Association of celiac disease and intestinal lymphomas and other cancers
Article Outline
Celiac
disease (CD) is associated with intestinal lymphoma and other forms of
cancer, especially adenocarcinoma of the small intestine, of the
pharynx, and of the esophagus. Enteropathy-associated T-cell lymphoma
(EATL) is a rare form of high-grade, T-cell non-Hodgkin lymphoma (NHL)
of the upper small intestine that is specifically associated with CD.
This NHL subtype arises in patients with either previously or
concomitantly diagnosed CD. In a subgroup of patients, there is
progressive deterioration of a refractory form of CD. EATL derives from a
clonal proliferation of intraepithelial lymphocyte s(IEL) and is often
disseminated at diagnosis. Extraintestinal presentations are not
uncommon in the liver/spleen, thyroid, skin, nasal sinus, and brain. The
outlook of EATL is poor. Recent studies indicated that
(1) CD is associated with a significantly increased risk for NHL, especially of the T-cell type and primarily localized in the gut (EATL);
(2) the CD-lymphoma association is less common than previously thought, with a relative risk close to 3;
(3) CD screening is not required in patients with NHL of any primary site at the onset, unless suggested by specific findings (T-cell origin and/or primary gut localization). The risk of NHL associated with clinically milder (or silent) forms could be lower than in typical cases of CD. Several follow-up studies suggest that the GFD protects from cancer development, especially if started during the first years of life. Strict adherence to the GFD seems to be the only possibility of preventing a subset of rare but very aggressive forms of cancer.
(1) CD is associated with a significantly increased risk for NHL, especially of the T-cell type and primarily localized in the gut (EATL);
(2) the CD-lymphoma association is less common than previously thought, with a relative risk close to 3;
(3) CD screening is not required in patients with NHL of any primary site at the onset, unless suggested by specific findings (T-cell origin and/or primary gut localization). The risk of NHL associated with clinically milder (or silent) forms could be lower than in typical cases of CD. Several follow-up studies suggest that the GFD protects from cancer development, especially if started during the first years of life. Strict adherence to the GFD seems to be the only possibility of preventing a subset of rare but very aggressive forms of cancer.
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