Abstract Details

Title Intravascular Lymphoma of the Central Nervous System: A Case Series Describing Clinical and Radiographic Characteristics

Topic Neuro-oncology

Presentation(s) P4 - Poster Session 4 (5:30 PM-6:30 PM)

Poster/Presentation
Number 13-015

Objective To characterize the common clinical and radiographic characteristics of intravascular lymphoma (IVL) in order to enable clinicians to pursue appropriate early diagnostics for this rare yet treatable entity.

Background IVL is a rare neoplastic disorder characterized by growth of malignant B-cells within the vasculature. IVL most commonly affects the brain and skin, and central nervous system manifestations are heterogeneous and challenging to recognize.

Design/Methods A retrospective chart review was conducted, collecting clinical, laboratory and radiographic data from a convenience sample of 5 patients with biopsy-proven intravascular lymphoma involving the central nervous system at the University of California San Francisco between 2013 and 2019.

Results 3 of 5 patients were female; median age at diagnosis was 64 years (Interquartile range 52-70). IVL was diagnosed by brain biopsy in 4 patients and by skin biopsy in 1. Cerebrospinal fluid (CSF) was non-inflammatory in 4 patients and 1 demonstrated lymphocytic pleocytosis; none had abnormal cytology or flow cytometry. All patients had elevated serum lactate dehydrogenase (LDH) at presentation. The 2 patients with lipid panels both had low high density lipoprotein (HDL) (less than 20 mg/dL). All patients had white matter hyperintensities and abnormal susceptibility on magnetic resonance imaging (MRI), plus at least one of the following: diffusion restriction, grey matter involvement, and gadolinium enhancement. Of the 4 patients with spine imaging, 2 demonstrated disease involvement of the cord.

Conclusions IVL has heterogeneous clinical and radiographic features but common MRI abnormalities include abnormal susceptibility and white matter T2 hyperintensities. High LDH, low HDL or MRI showing diffusion restriction, enhancement, and grey matter involvement may be supportive. Random skin biopsy provides an attractive alternative for diagnosis of IVL in the appropriate clinical scenario.

Authors/Disclosures
Megan Richie, MD (University of California, San Francisco) PRESENTER	Dr. Richie has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for JAMA Neurology.
Maulik Shah, MD (UCSF Neurology)	Dr. Shah has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for National Football League.
Elan Guterman, MD (University of California, San Francisco)	Dr. Guterman has received personal compensation in the range of $5,000-$9,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for JAMA Neurology. Dr. Guterman has stock in REMO Health. The institution of Dr. Guterman has received research support from NIH. The institution of Dr. Guterman has received research support from AAN.

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