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On 2nd August 2017, in Histopathology, Victoria Alegría-Landa from Fundación Jiménez Díaz, Universidad Autónoma, Madrid, Spain, et al. published a case report of a Caucasian male, 81 years of age, with Cutaneous Natural Killer (NK)/T-cell Intravascular Lymphoma (IVL).
IVL is rare and the majority of cases are a type of extranodal Diffuse Large B-Cell Lymphoma (DLBCL). Less than 10% of reported cases are of T-cell origin. This current case of cutaneous NK/T-cell IVL with cytotoxic immunophenotype and EBV positivity is the first triple negative (TCR-βF1, TCR-γ, and CD56) case to be described. This entity has not yet been classified by the World Health Organization and the authors urge that is should be considered as a distinct subtype in future classifications.
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 WHO classification of lymphoproliferative disorders. We describe a case of cutaneous NK/T IVL, with a cytotoxic immunophenotype and EBV positivity. However, our case was immunohistochemically negative not only for 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 the recognition of this NK/T-cell lineage intravascular lymphoma due to its particular immunophenotypic 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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