Track

Case Reports

Abstract

Intravascular NK/T-cell lymphoma (IVNKTL) is an exceptionally rare, aggressive, EBV-associated lymphoma characterized by neoplastic NK/T-cells confined to small- and medium-sized vessel lumina. Since its first description in 2003, only 27 well-documented cases have been reported. We describe a 34-year-old woman with minimal medical history who presented with dizziness, a fall, and cerebellar ischemic stroke. Workup revealed heterozygous Factor V Leiden mutation, CSF lymphocytic pleocytosis, and negative autoimmune studies. A faint rash, present from the outset, was attributed to bruising. Weeks later, she re-presented with vertigo, diaphoresis, and nausea. MRI showed leptomeningeal enhancement and multifocal T2 hyperintensities in the thalamus, frontal cortex, and cerebellum. Repeat CSF remained negative for infection and malignancy. Suspected CNS vasculitis prompted cerebellar and meningeal biopsies, revealing intravascular  atypical lymphoid cells (CD3ε+/CD56+/granzyme B+/EBER+), diagnostic of IVNKTL. Skin biopsy of a bruise-like area showed dilated dermal vessels with early thrombus admixed with similar malignant (CD3+/CD56+/EBER+/TIA1+) lymphocytes. Her condition rapidly deteriorated, and she died prior to completing chemotherapy. This case underscores that IVNKTL can occur in young adults, mimic CNS vasculitis, and present with nonspecific cutaneous lesions early in the disease. Concurrent CNS and skin involvement carries a worse prognosis than single-organ disease. Distinguishing IVNKTL from intravascular large B-cell lymphoma (CD3-/CD20+) is essential, given its EBV association and differing biology. Given the paucity of reported cases and poor prognosis, greater awareness is critical for early recognition. Comprehensive clinicopathologic studies are needed to better define epidemiology, pathogenesis, and optimal management strategies for IVNKTL.