There are a number of rare T-cell lymphoma subtypes that may be encountered in clinical practice. In recent years, improved immunohistochemical techniques and molecular tumor profiling have permitted refinement of some of the diagnostic categories in this group, as well as the recognition of distinct conditions not previously well elucidated. In this chapter, we cover the diagnostic and clinical features of some of the more common of these conditions, including subcutaneous panniculitis-like T-cell lymphoma, cutaneous gamma-delta T-cell lymphoma, enteropathy-associated T-cell lymphoma, monomorphic epitheliotropic intestinal T-cell lymphoma, primary cutaneous CD8-positive aggressive epidermotropic cytotoxic T-cell lymphoma, CD4-positive small/medium T-cell lymphoproliferative disorder, and acral CD8-positive T-cell lymphoma. Given the rarity of these conditions, optimal treatments approaches are not always well established, not least as data from large-scale clinical trials are lacking. In this chapter, we aim to provide a summation of current thinking around best treatment, as well as highlighting some controversies in the management of these diagnoses.
Keywords: Acral CD8-positive T-cell lymphoma; CD4-positive small/medium T-cell lymphoproliferative disorder; Cutaneous gamma-delta T-cell lymphoma; Enteropathy-associated T-cell lymphoma; Monomorphic epitheliotropic intestinal T-cell lymphoma; Primary cutaneous CD8-positive aggressive epidermotropic cytotoxic T-cell lymphoma; Primary cutaneous T-cell lymphoma; Subcutaneous panniculitis-like T-cell lymphoma.