Non-cytotoxic gamma-delta peripheral T-cell lymphoma affecting the mandibular and parotidal lymph nodes and the skin.
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.
Lima
Margarida
M
Serviço de Hematologia, Unidade de Citometria, Hospital Geral de Santo António, Rua D Manuel II, s/n 4099-001 Porto, Portugal. mmc.lima@clix.pt
Canelhas
Aurea
A
Santos
Carlos
C
dos Anjos Teixeira
Maria
M
Coutinho
Jorge
J
Alves
Rosário
R
Queirós
Maria Luís
ML
Fonseca
Sónia
S
Santos
Ana Helena
AH
Gonçalves
Vicente
V
Massa
Antonio
A
Justiça
Benvindo
B
D000951:Antigens, Neoplasm / Q000032:analysis; D014408:Biomarkers, Tumor / Q000032:analysis; D003937:Diagnosis, Differential; D017809:Fatal Outcome; D005260:Female; D053804:Granzymes; D006801:Humans; D016130:Immunophenotyping; D008198:Lymph Nodes / Q000737:chemistry / Q000473:pathology*; D016410:Lymphoma, T-Cell, Cutaneous / Q000175:diagnosis / Q000473:pathology*; D016411:Lymphoma, T-Cell, Peripheral / Q000175:diagnosis / Q000473:pathology*; D008562:Membrane Glycoproteins / Q000032:analysis; D008875:Middle Aged; D009333:Neck; D009363:Neoplasm Proteins / Q000032:analysis; D014411:Neoplastic Stem Cells / Q000473:pathology; D054353:Perforin; D010641:Phenotype; D052899:Pore Forming Cytotoxic Proteins; D016692:Receptors, Antigen, T-Cell, gamma-delta / Q000032:analysis*; D012697:Serine Endopeptidases / Q000032:analysis; D012878:Skin Neoplasms / Q000737:chemistry / Q000175:diagnosis / Q000473:pathology*; D016176:T-Lymphocyte Subsets / Q000473:pathology
D000951:Antigens, Neoplasm; D014408:Biomarkers, Tumor; D008562:Membrane Glycoproteins; D009363:Neoplasm Proteins; D052899:Pore Forming Cytotoxic Proteins; D016692:Receptors, Antigen, T-Cell, gamma-delta; D054353:Perforin; C509213:GZMB protein, human; D053804:Granzymes; D012697:Serine Endopeptidases