Definition
A neoplasm of intra-epithelial T-lymphocytes
The disease is uncommon, but is of increasing incidence in parts of the world with a high prevalence of coeliac disease.
Most patients have a history of coeliac disease, from childhood, more commonly of adult onset, sometimes concomitant with he onset of the lymphoma. There may be a prodromal period of refractory coeliac disease (ulcerative jejunitis). They present with abdominal pain, sometimes with intestinal perforation.
The small intestine shows multiple ulcerating masses, sometimes a large exophytic mass.
Cytomorphology is variable. The neoplastic cells are most commonly medium size and monomorphic. Less often they are highly pleomorphic, resembling anaplastic large cell lymphoma. There may be a heavy infiltrate of histiocytes an eosinophils, which may obscure the neoplastic cells. The adjacent mucosa shows enteropathy-associated villous atrophy.
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positive |
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positive |
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negative |
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negative |
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positive |
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variable |
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most cases show some positive cells |
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some |
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CD103 |
positive |
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TCR |
usually ab positive |
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positive |
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granzyme B |
positive |
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perforin |
positive |
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EBV |
usually negative |
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The lymphocytes in the adjacent enteropathic mucosa are CD3+, CD4-, CD5-, CD8-.
†: fresh frozen tissue only
extranodal NK/T-cell lymphoma, nasal type, which may also involve the gastrointestinal tract.
Usually poor.
World Health Organization Classification of Tumours, Tumours of the haematopoietic and lymphoid tissues, IARC Press 2001.
1Delsol G, New antibodies and new applications of old antibodies in the diagnosis of hematolymphoid neoplasms. In Immunohistochemistry Long Course, Nice 1998.
This page last revised 19.12.2002.
©SMUHT/PW Bishop