Gamma/delta T-cell lymphoma with mycosis fungoides-like clinical course transforming to "TCR-silent" aggressive lymphoma: description of one case.

Primary cutaneous γδ T-cell lymphomas (PCGDTL) are a heterogeneous group of lymphomas representing about 1% of primary cutaneous T-cell lymphomas (CTCL) and mostly regarded as clinically aggressive. Current WHO-EORTC classification recognizes different clinic-pathologic subsets of PCGDTL, but it suggests that cases showing a mycosis fungoides (MF)-like clinical presentation and histopathology should be classified as MF irrespective of phenotype for their indolent course. Herein, we describe a case of γδ-MF, featuring at onset a granulomatous pattern, with subsequent clinical worsening signaled by the development of an ulcero-necrotic lesion and systemic dissemination, leading to death in 5 months. Clinical progression was sustained by a shift to mature Tcell lymphoma composed of medium to large-sized blastoid T-cells featuring a TCR silent immunophenotype. This article is protected by copyright. All rights reserved.