Gamma/delta T‐cell lymphoma with mycosis fungoides‐like clinical course transforming to 'T‐cell‐receptor‐silent' aggressive lymphoma: Description of one case
| Autor: | Annamaria Hotz, Fabrizio Ciambelli, Stefania Cione, Giorgio Alberto Croci, Caterina Cecchetti, Filippo Crivelli, Dario Tomasini |
|---|---|
| Rok vydání: | 2021 |
| Předmět: |
Pathology
medicine.medical_specialty Mycosis fungoides Histology biology business.industry Cutaneous T-cell lymphoma T-cell receptor Aggressive lymphoma Dermatology medicine.disease Blastoid biology.organism_classification Pathology and Forensic Medicine Lymphoma 030207 dermatology & venereal diseases 03 medical and health sciences 0302 clinical medicine medicine.anatomical_structure Immunophenotyping 030220 oncology & carcinogenesis medicine Gamma delta T cell business |
| Zdroj: | Journal of Cutaneous Pathology. 48:1197-1203 |
| ISSN: | 1600-0560 0303-6987 |
| DOI: | 10.1111/cup.14063 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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