Cutaneous intravascular natural killer/T cell lymphoma with peculiar immunophenotype |
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| Authors: | Victoria Alegría‐Landa Félix Manzarbeitia Maria G Salvatierra Calderón Luis Requena Socorro M Rodríguez‐Pinilla |
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| Affiliation: | 1. Department of Dermatology, Fundación Jiménez Díaz, Universidad Autónoma, Madrid, Spain;2. Department of Pathology, Fundación Jiménez Díaz, Universidad Autónoma, Madrid, Spain;3. Department of Haematology, Hospital Universitario Rey Juan Carlos, Universidad Rey Juan Carlos, Móstoles, Madrid, Spain |
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| Abstract: | 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 CD56, 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. |
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| Keywords: | cytotoxic markers intravascular lymphoma NK/T immunophenotype TCR‐β F1 TCR‐γ and CD56 negativity |
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