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A new type of intestinal lymphoma

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Enteropathy-type intestinal T cell lymphoma (EITCL) is a known complication of chronic and untreated coeliac disease. Often diagnosed late, it is then difficult to treat. But new research suggests there is a reliable diagnostic clue that could improve the chances of earlier detection.

Clonal T cell receptor (TCR-γ) gene rearrangments and loss of T cell antigens, such as CD8 and T cell receptor β (TC R-β), have been found in some patients with refractory sprue, which might be an early form of EITCL. So the authors looked for similar evidence in the duodenal biopsy tissue of 32 patients with different forms of refractory sprue, EITCL, and coeliac disease.

They found TCR-γ gene rerrangement clonality in three out of eight patients with overt EITCL, in two out of three with refractory sprue evolving into EITCL, and in one out of six with refractory sprue of unknown cause. They also found the rearrangement in both patients with jejunitis, but not in the 13 patients with either refractory sprue of known cause or with coeliac disease.

Immunohistology was highly specific for T cell antigen loss (77%) and clonality (100%) in EITCL. Sensitivity reached 62 per cent for both CD8 loss and proliferation, but 100 per cent for TCR-β loss.

Intraepithelial lymphocytes displayed an abnormal phenotype in all cases of EITCL and in all but one with evidence of underlying coeliac disease. And the authors conclude that this finding in refractory sprue indicates early EITCL, which they name “sprue-like intestinal T cell lymphoma.”

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