Disseminated CNS Intravascular T-cell Lymphoma Masquerading as CNS Vasculitis and Stroke
Justin Lei1, Sarah Rathman3, Swathi Nagabushan4, Mehmood Salim2, Ayman Al-Salaimeh1, Muhammad-Atif Zubairi3
1Neurology, UCF College of Medicine, 2UCF College of Medicine, 3UCF/HCA Osceola Hospital, 4.
Objective:
To report a rare case of disseminated intravascular T-cell lymphoma with central nervous system (CNS) involvement presenting as acute ischemic stroke and mimicking CNS vasculitis
Background:
Intravascular lymphoma (IVL) is a rare malignancy characterized by intraluminal proliferation of neoplastic lymphocytes within small and medium-sized vessels. In the rare cases of IVL spread to the CNS, the neurological manifestations are varied and often devastating. While most IVL cases are of B-cell lineage, intravascular T-cell lymphoma is exceedingly rare and aggressive. Diagnosis is challenging due to its nonspecific clinical and radiographic features and the need for invasive techniques to diagnose with certainty such as brain biopsy.
Design/Methods:
Case presentation and review of the literature.
Results:
53-year-old man with a history of gastrointestinal T-cell lymphoma refractory to treatment, central diabetes insipidus, and prior small-vessel cerebrovascular accident presented to the emergency department with acute right lower extremity weakness following focal seizure-like activity. CT angiography revealed left A1 occlusion with multifocal arterial irregularities. The patient received Tenecteplase for presumed acute ischemic stroke which was complicated by hemorrhagic conversion. Digital subtraction angiography showed multifocal vessel wall irregularities and stenoses suggestive of CNS vasculitis. CSF analysis revealed pleocytosis, hypoglycorrhachia, and significantly elevated protein. Cytology demonstrated malignant lymphoid cells, and flow cytometry identified an atypical T-cell population (CD3+, CD5+, CD2+, loss of CD7), confirming intravascular CNS lymphoma. The patient declined brain biopsy and further oncologic therapy, prioritizing comfort care.
Conclusions:
Disseminated intravascular T-cell lymphoma can closely mimic CNS vasculitis and cause strokes. Diagnosis requires integration of multiple diagnostics when tissue biopsy is unavailable. Early endocrine abnormalities, such as central diabetes insipidus, may precede even the discovery of the cancer itself. This case also underscores the importance of shared decision-making and aligning management with patient preferences in advanced neuro-oncologic disease.
Disclosure: Dr. Lei has nothing to disclose. Dr. Al-Salaimeh has nothing to disclose.
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