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

Abstract

BackgroundIntravascular 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 presentationA 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.ConclusionsThe 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.

Primary Pulmonary B-Cell Lymphoma: A Review and Update

“…CD34 immunostaining for endothelial cells may be useful in challenging cases, although this marker is not present in routine panels of antibodies [ 3 ]. Although very rare, some forms of intravascular lymphomas of T-cell origin have been reported [ 159 ].…”

Intravascular pseudolymphomatous angiosarcoma: A new finding potentially mistaken for intravascular lymphoma

“…The differential in the current case would also include intravascular lymphoma. There are several types of intravascular lymphoma, including T‐cell, 4 B‐cell, 5 and NK/T‐cell 6,7 . Of these, the best established is intravascular large B‐cell lymphoma (IVLBCL).…”

“…These lymphomas are all characterized by an intravascular proliferation of neoplastic lymphocytes. In general, intravascular lymphomas have a poor prognosis and present with myriad neurologic and constitutional symptoms 4‐6 . Diagnosis is based on immunohistochemical staining patterns; T‐cell intravascular lymphoma stains for CD3, IVLBCL stains for CD20, and NK/T‐cell for stains for CD56.…”

EBV-Driven Lymphoproliferative Disorders and Lymphomas of the Gastrointestinal Tract: A Spectrum of Entities with a Common Denominator (Part 2)

“…Intravascular lymphoma is more often of B-cell lineage and, rarely, EBV-positive, whereas the rare cases of IV lymphoma of NK/T cell origin are more often EBV-associated. Skin and CNS are more frequently involved, although any site can be affected [ 84 ].…”