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Gastrointestinal intraepithelial lymphocytes and T cell lymphomas
  1. I N Farstad1,
  2. K E A Lundin2
  1. 1Institute of Pathology, University of Oslo, Rikshospitalet, Oslo, Norway
  2. 2Department of Medicine, University of Oslo, Rikshospitalet, Oslo, Norway
  1. Correspondence to:
    Dr I N Farstad, Institute of Pathology, Rikshospitalet, N-0027 Oslo, Norway;
    i.n.farstad{at}labmed.uio.no

https://doi.org/10.1136/gut.52.2.163

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    Enteropathy-type intestinal T cell lymphoma is assumed to derive from intraepithelial lymphocytes and recent reports describing an intermediate or “cryptic” stage of this lymphoma in patients with many characteristics of coeliac disease have challenged aspects of our understanding of coeliac disease and lymphoma development

    Gastrointestinal T cell lymphomas occur less often than those of B cells but have a much more unfavourable prognosis.1 Enteropathy-associated T cell lymphoma (EATL) is the predominant subtype,1 “enteropathy” indicating a link to coeliac disease (CD).1 More recently, enteropathy-type intestinal T cell lymphoma (EITCL) seems to be the preferred term for this entity.2,3 EITCL is assumed to derive from intraepithelial lymphocytes (IELs) and recent reports describing an intermediate or “cryptic” stage of this lymphoma in patients with many characteristics of CD have provided a “missing link” in our understanding of the pathogenesis of EITCL.4,5

    Refractory coeliac sprue (RCS) is diagnosed in patients with CD-like enteropathy who do not respond to a gluten free diet, primarily or some time after an initial response.6 RCS patients with phenotypically normal and polyclonal IELs may respond to immunosuppressive therapy.5 However, a large fraction of RCS patients have heavily increased IEL numbers lacking surface CD3 (cytoplasmic ε chains being present) and usually also CD8, in addition to being monoclonal.5,6 Loss of …

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