Intravascular NK/T-cell lymphoma, Epstein-Barr virus positive with multiorgan involvement: A clinical dilemma

Magda Zanelli, Maria Cecilia Mengoli, Rachele Del Sordo, Angelo Cagini, Loredana De Marco, Edoardo Simonetti, Giovanni Martino, Maurizio Zizzo, Stefano Ascani

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Abstract

Background: Intravascular lymphoma is a rare type of non-Hodgkin lymphoma mostly of B-cell lineage. A few cases of intravascular lymphoma have been found to be of NK/T-cell origin, mainly affecting the skin and central nervous system. Case presentation: A 54-year-old Caucasian man sought care because of a 2 weeks history of jaundice and intermittent fever, not responsive to antibiotics and antipyretics. Laboratory tests showed low blood oxygen concentration and pancytopenia. Serum microbiological tests were negative. Computerized tomography (CT) scan revealed hepatosplenomegaly and diffuse ground-glass opacities in both lungs without interlobular septal thickening. Despite oxygen therapy, the clinical conditions rapidly deteriorated leading to death 3 days after admission. Autopsy revealed a multiorgan involvement by an Epstein-Barr virus positive NK/T-cell lymphoma, strikingly growing within the blood vessel lumina, in absence of skin lesions. Conclusions: The current case highlights the pathological features of this rare entity, the protean clinical presentation of which is often misleading, resulting in delayed diagnosis and treatment.

Original languageEnglish
Article number1115
JournalBMC Cancer
Volume18
Issue number1
DOIs
Publication statusPublished - Nov 15 2018

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Keywords

  • Epstein-Barr virus
  • Intravascular
  • Lymphoma
  • Natural killer cell
  • T-cell

ASJC Scopus subject areas

  • Oncology
  • Genetics
  • Cancer Research

Cite this

Zanelli, M., Mengoli, M. C., Del Sordo, R., Cagini, A., De Marco, L., Simonetti, E., Martino, G., Zizzo, M., & Ascani, S. (2018). Intravascular NK/T-cell lymphoma, Epstein-Barr virus positive with multiorgan involvement: A clinical dilemma. BMC Cancer, 18(1), [1115]. https://doi.org/10.1186/s12885-018-5001-6