Rapidly progressive varicella zoster virus vasculopathy in a chemotherapy- and steroid-immunosuppressed patient with refractory diffuse large B-cell lymphoma: diagnostic and therapeutic challenges

TL;DR

A rare and severe neurological complication caused by the varicella zoster virus in an immunosuppressed cancer patient is described, highlighting diagnostic challenges and the need for early detection.

Contribution

This case report emphasizes the diagnostic complexity of VZV vasculopathy in immunocompromised patients and the potential value of vessel wall MRI in distinguishing it from similar conditions.

Findings

VZV vasculopathy can rapidly progress in immunosuppressed patients, leading to fatal outcomes despite treatment.

CSF evaluation is crucial for early diagnosis of VZV vasculopathy.

Vessel wall MRI may help differentiate VZV vasculopathy from RCVS.

Abstract

Varicella zoster virus (VZV) vasculopathy is a rare but severe neurological complication that frequently results in a reversible cerebral vasoconstriction syndrome (RCVS). Diagnosis is often delayed because early manifestations are nonspecific and radiologic findings often overlap with those of other cerebrovascular disorders. We describe the fatal case of a heavily pretreated 60-year-old man with refractory diffuse large B-cell lymphoma undergoing evaluation for chimeric antigen receptor (CAR) T-cell therapy. The patient developed multifocal intracranial arterial stenosis and an acute infarction during chemotherapy-induced immunosuppression. The initial radiological features suggested RCVS; however, cerebrospinal fluid (CSF) analysis using polymerase chain reaction (PCR) and anti-IgG antibodies confirmed the presence of VZV. Despite intravenous acyclovir and high-dose corticosteroids, the vasculopathy progressed rapidly, resulting in recurrent infarctions, seizures, and death. This case highlights the diagnostic complexity of VZV vasculopathy in immunocompromised patients and underscores the importance of early CSF evaluation, heightened clinical vigilance for opportunistic viral complications, and the potential diagnostic value of vessel wall magnetic resonance imaging (MRI) in differentiating VZV vasculopathy from mimicking conditions such as RCVS.

The online version contains supplementary material available at 10.1007/s44313-026-00126-5.

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