Intravascular Lymphoma: A Subtype of B-cell Lymphoma as a Rare Cause Of Recurrent Strokes
Elisha Jindal1, Chad Sullivan2, Nooshin Kiani Nia3, Karin Olds4, Jon Wilson5
1Saba University School Of Medicine, 2University of Missouri Kansas City, 3Neurology, University of Missouri Kansas City, 4Neurology, Saint Luke's Marion Bloch Neuroscience Institute, 5Neuropathology, Arkana Laboratories
Objective:
Expanding the standard testing protocols for recurrent strokes allows for potentially treatable diagnoses. Our purpose is to discuss a case of intravascular lymphoma (IVL) presenting as a rare cause of recurrent strokes.
Background:
IVL is a rare subtype of B-cell-lymphoma involving the invasion of small and medium-sized vasculature lumina, typically presenting with neurological or cutaneous symptoms. Neurological symptoms include recurring strokes, myelopathy, encephalopathy, vertigo, dementia, and sensory or motor deficits. An important presentation to note is the occurrence of isolated strokes within a few months. Magnetic-Resonance-Imaging (MRI) findings vary from infarct-like lesions, non-specific white matter changes, meningeal enhancement, or focal hyperintensities with no pathognomonic difference between embolic strokes and IVL. However, skin biopsies have been reported to be highly sensitive in suspected IVL patients and may be considered in patients with recurrent strokes despite optimal antiplatelet and anticoagulation therapy.
Design/Methods:
We are presenting a 66-year-old female with recurrent strokes over the course of three to four-months from the initial presentation despite stroke prevention measures. Her blood vessel imaging was not supportive of atherosclerotic disease, cardiogenic emboli, or vasculitis; infectious and autoimmune workup were negative. As a result, based on her MRI findings, alternative diagnoses such as IVL and Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) were considered. A muscle biopsy was obtained that was consistent with B-cell lymphoma. Consequently, the patient was started on an appropriate chemotherapy regimen.
Conclusions:
IVL is a subtype of B-cell lymphoma characterized by proliferation within the vascular system. This proliferation occurs without clear evidence of circulating cells in the peripheral blood, particularly the lumina of capillaries and post-capillary venules; this prolongs diagnosis based on traditional diagnostic methods. IVL may present with neurological involvement of the central nervous system that can come in the context of recurrent strokes, rapidly progressing dementia, and peripheral neuropathy.