Guidelines on Diagnosis and Treatment of Malignant Lymphomas

Recommended Investigations
Evaluation of patients with refractory coeliac disease
Most patients with EATCL have a prior history of coeliac disease
or simultaneous diagnosis of underlying coeliac disease. There is
currently no recommendation for routine surveillance of coeliac
disease patients who have responded to a gluten free diet.
Refractory coeliac disease and ulcerative jejunitis probably
represent a pre-neoplastic condition with frequent evolution
to clonal disease and associated phenotypic changes. For these
reasons a high level of suspicion in patients with coeliac disease
with persistent symptoms is necessary. Immunophenotyping
of the intraepithelial lymphocytes in serial biopsies in these
patients may be useful in detecting phenotypic change
(evolution to CD3+, CD8-, CD4- phenotype).
Patients with established diagnosis of lymphoma
Investigations should be carried out as for other high grade
lymphomas. In addition, a small bowel follow through should
be performed, and nutritional status assessed. Clonal T-cell
populations may be identified in the peripheral blood, but
the clinical significance of this is unclear.
Treatment
Standard treatment with anthracycline containing combination
chemotherapy is usually used despite poor results. The
Nottingham group have pioneered an approach using IEV
chemotherapy (2 cycles) followed by 2 courses of Methotrexate
3gms/m2 for CNS prophylaxis followed by an autologous PBSCT
with survival of 4 of 6 patients beyond 2 years. Gemctabine
based therapy may be useful in aggressive T-NHLs such as
EATCL. Perforation may occur during initial chemotherapy.
Patients often require parenteral nutrition.
In patients with refractory coeliac disease or ulcerative jejunitis,
consideration may be given to pre-emptive treatment with
combination chemotherapy if phenotypic change in the
intraepithelial lymphocytes or clonally rearranged T-cells are
detected, but this remains an experimental approach..
Response Evaluation and Follow Up
Re-evaluation at intervals should include endoscopic evaluation,
small bowel follow through and small bowel biopsy.
Prognostic Factors / Index
EATCL has a poor prognosis with a median survival of
approximately 8 months and one year failure free survival
of less than 20%.
Potential Pitfalls
Failure to recognise the development of EATCL in patients
with refractory coeliac disease or ulcerative jejunitis. Frequent
surveillance with endoscopic biopsy and immunophenotyping
is recommended in patients with persistent or suspicious
symptoms, and in those with refractory coeliac disease or
with ulcerative jejunitis.
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