Intravascular lymphoma mimicking vasculitis

Highlights

• Intravascular lymphoma can present with neurologic symptoms.

• The tumor may clinically mimic vasculitis.

• Diagnosis requires a biopsy sample to establish.

Abstract

Intravascular lymphoma is a rare malignancy which is characterized by a proliferation of atypical appearing B cells, generally confined to vascular lumina. A tissue biopsy demonstrating the pathology is required to make a diagnosis. The tumor is often disseminated at the time of diagnosis and prognosis is poor, even with aggressive chemotherapy. Neurologic presentations of this neoplasm can be quite varied. This report documents the presence of intravascular lymphoma diagnosed on a brain biopsy in a 60-year-old man. He initially presented 6 months before brain biopsy with chest pain and hypotension, warranting coronary artery bypass graft surgery. Four months later, he presented with signs attributed to a stroke (diaphoresis, slumped over in a chair and left hand weakness). He subsequently developed a sudden onset wide-based gait, left leg numbness, word finding difficulties and worsening confusion. A MRI study showed multiple infarcts in the brain, including cerebellum. Invasive angiogram suggested vasculitis. He was started on a course of treatment for presumed central nervous system vasculitis. He continued to develop signs suggestive of ongoing infarct development and a biopsy from the right parietal was taken. The biopsy showed atypical intravascular CD20 positive staining B cells, consistent with intravascular lymphoma.

Introduction

Intravascular lymphoma (angiotropic large cell lymphoma) is a well-recognized, albeit rare, aggressive systemic B cell lymphoma with an annual incidence of 0.5 cases per 1,000,000 population [1]. The tumor is marked by the proliferation of lymphomatous cells within blood vessel lumina. An absence of CD29 and CD54 surface ligands may be responsible for confinement of tumor cells to blood vessels [2]. Neurologic symptoms are fairly common in affected patients. One meta-analysis study reviewing 654 patients, noted evidence of neurologic complications in 52% of patients [2]. The current case report presents a case of intravascular lymphoma diagnosed on brain biopsy in a 60-year-old man who clinically was thought to have vasculitis.