Abstract
In contrast to the majority of alphabeta peripheral T cell lymphomas (PTCL), which usually originate in lymph nodes and do not express NK-associated molecules, most gammadelta PTCL express a cytotoxic phenotype and originate at extranodal sites. We report a case of a patient with a gamma-delta PTCL who presented with large mandibular and parotidal lymphadenopathy and skin lesions. CD3(+)/TCR-Vdelta1 (+) lymphoma cells did not express the cell surface (CD11b, CD11c, CD16, CD56 and CD57) and cytoplasmic granule molecules (Perforin and Granzyme B) that usually characterize the cytotoxic T-cells, a phenotype that fulfils the criteria for diagnosis of a rare non-cytotoxic variant of a gammadelta T-cell lymphoma. "In situ" hybridization for Epstein-Barr virus-encoded RNA and latent membrane protein-1 gave negative results. The disease had an aggressive course and was resistant to chemotherapy and the patient died 4 months after diagnosis.
MeSH terms
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Antigens, Neoplasm / analysis
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Biomarkers, Tumor / analysis
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Diagnosis, Differential
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Fatal Outcome
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Female
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Granzymes
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Humans
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Immunophenotyping
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Lymph Nodes / chemistry
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Lymph Nodes / pathology*
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Lymphoma, T-Cell, Cutaneous / diagnosis
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Lymphoma, T-Cell, Cutaneous / pathology*
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Lymphoma, T-Cell, Peripheral / diagnosis
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Lymphoma, T-Cell, Peripheral / pathology*
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Membrane Glycoproteins / analysis
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Middle Aged
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Neck
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Neoplasm Proteins / analysis
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Neoplastic Stem Cells / pathology
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Perforin
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Phenotype
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Pore Forming Cytotoxic Proteins
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Receptors, Antigen, T-Cell, gamma-delta / analysis*
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Serine Endopeptidases / analysis
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Skin Neoplasms / chemistry
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Skin Neoplasms / diagnosis
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Skin Neoplasms / pathology*
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T-Lymphocyte Subsets / pathology
Substances
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Antigens, Neoplasm
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Biomarkers, Tumor
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Membrane Glycoproteins
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Neoplasm Proteins
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Pore Forming Cytotoxic Proteins
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Receptors, Antigen, T-Cell, gamma-delta
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Perforin
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GZMB protein, human
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Granzymes
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Serine Endopeptidases