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Cutaneous intravascular natural killer/T cell lymphoma with peculiar immunophenotype
Author(s) -
AlegríaLanda Victoria,
Manzarbeitia Félix,
Salvatierra Calderón Maria G,
Requena Luis,
RodríguezPinilla Socorro M
Publication year - 2017
Publication title -
histopathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.626
H-Index - 124
eISSN - 1365-2559
pISSN - 0309-0167
DOI - 10.1111/his.13332
Subject(s) - immunophenotyping , lymphoma , pathology , natural killer cell , t cell receptor , t cell lymphoma , lymphoproliferative disorders , immunology , t cell , medicine , biology , cytotoxic t cell , antigen , immune system , biochemistry , in vitro
Intravascular lymphoma ( IVL ) is a rare entity. Most cases are a variant of extranodal diffuse large B cell lymphoma, and fewer than 10% of the published cases are of T cell origin. Only intravascular B cell lymphoma is recognized as a distinct entity in the most recent World Health Organization ( WHO ) classification of lymphoproliferative disorders. We describe a case of cutaneous natural killer ( NK )/T IVL , with a cytotoxic immunophenotype and Epstein–Barr virus ( EBV ) positivity. However, our case was immunohistochemically negative not only for T cell receptor ( TCR )‐βF1 and TCR ‐γ ( TCR ‐silent), but also for CD 56, making it the first triple‐negative NK /T IVL case to be described. We urge recognition of this NK /T cell lineage intravascular lymphoma due to its particular immunophenotypical profile and its unvarying relationship with EBV . Its occurrence should not be considered a coincidence, but rather a key aspect of the pathogenic background of this haematological neoplasm.