Enteropathy-type T-cell lymphoma

Definition

A neoplasm of intra-epithelial T-lymphocytes

Synonyms

Epidemiology

The disease is uncommon, but is of increasing incidence in parts of the world with a high prevalence of coeliac disease.

Clinical features

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.

Macroscopic appearances

The small intestine shows multiple ulcerating masses, sometimes a large exophytic mass.

Histopathology

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.

Epitheliotropic T-cell lymphoma is a variant in which the lymphocytes are small and show an intraepithelial distribution.

Immunohistochemistry

CD1a

   

CD2

positive

CD3

positive

CD4

negative

CD5

negative

CD7

positive

CD8

variable

CD11b

 

CD16

 

CD25

 

CD30

most cases show some positive cells

CD56

some

CD57

 

CD103

positive

TCR

usually ab positive

TdT

 

TIA-1

positive

granzyme B

positive

perforin

positive

ALK

 

EBV

usually negative

 

The lymphocytes in the adjacent enteropathic mucosa are CD3+, CD4-, CD5-, CD8-.

†: fresh frozen tissue only

Differential diagnosis

Prognosis

Usually poor.

References

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