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Gamma/delta T‐cell lymphoma with mycosis fungoides‐like clinical course transforming to “T‐cell‐receptor‐silent” aggressive lymphoma: Description of one case
Author(s) -
Tomasini Dario,
Croci Giorgio Alberto,
Hotz Annamaria,
Cione Stefania,
Cecchetti Caterina,
Ciambelli Fabrizio,
Crivelli Filippo
Publication year - 2021
Publication title -
journal of cutaneous pathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.597
H-Index - 75
eISSN - 1600-0560
pISSN - 0303-6987
DOI - 10.1111/cup.14063
Subject(s) - mycosis fungoides , immunophenotyping , lymphoma , medicine , pathology , blastoid , histopathology , aggressive lymphoma , cd5 , peripheral t cell lymphoma , t cell lymphoma , cutaneous lymphoma , cutaneous t cell lymphoma , t cell , immunology , mantle cell lymphoma , antigen , immune system , rituximab
Primary cutaneous γδ T‐cell lymphomas (PCGDTLs) are a heterogeneous group of lymphomas representing about 1% of primary cutaneous T‐cell lymphomas (CTCLs) 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 T‐cell lymphoma composed of medium to large‐sized blastoid T‐cells featuring a T‐cell receptor (TCR) silent immunophenotype.