Treatment of Indolent T-Cell Lymphoma of the Gastrointestinal Tract

In contrast to the more aggressive forms of T-cell lymphoma with intestinal involvement, indolent T-cell lymphoma of the gastrointestinal tract (iTCL-GI) often presents more indolently. iTCL-GI can be difficult to distinguish from inflammatory bowel disease or other autoimmune disorders with gastrointestinal involvement.[1,2]

iTCL-GI is often diagnosed when multiple biopsies from different sites show matching clones by T-cell receptor gene rearrangement testing. While the immunophenotype can vary (iTCL-GI is frequently CD4+ but can also be CD8+ or CD4-/CD8-), Ki-67 is typically very low at 5% to 10%. Unlike some other intestinal lymphomas, Epstein-Barr encoding region staining for Epstein-Barr virus is usually negative. Although initial reports of this rare entity suggested a low likelihood of transformation to a more aggressive process, transformation to peripheral T-cell lymphoma, not otherwise specific and other aggressive forms has since been documented.[3]