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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
- Source :
- Journal of Cutaneous Pathology. 48:1197-1203
- Publication Year :
- 2021
- Publisher :
- Wiley, 2021.
-
Abstract
- 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.
- Subjects :
- 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
Subjects
Details
- ISSN :
- 16000560 and 03036987
- Volume :
- 48
- Database :
- OpenAIRE
- Journal :
- Journal of Cutaneous Pathology
- Accession number :
- edsair.doi...........6d1916ca32b6785caf7c677a13202942
- Full Text :
- https://doi.org/10.1111/cup.14063