Abstract Details

Title Clinical Spectrum and Course in Individuals with Intravascular Large B-Cell Lymphoma Affecting the Nervous System: A Case Series

Topic Neuro-oncology

Presentation(s) P01 - (-)

Poster/Presentation
Number 102

Objective

Background IVLBL is a rare, aggressive subtype of extranodal large B-cell lymphoma affecting nervous the system and characterized by diffuse proliferation of atypical B-lymphocytes within lumina of small vessels. It presents with heterogeneous clinical manifestations that can mimic more than one pathogenic entity. A bewildering array of clinical symptoms, non-specific imaging findings, and a rapid, often fatal, clinical course make early clinical diagnosis extremely challenging, thus delaying treatment.

Design/Methods We cared for five patients with intravascular lymphoma affecting central or peripheral nervous system and reviewed clinical, laboratory, neuro-imaging, and pathological data and treatment outcomes.

Results All but one patient were Caucasian (3 women and 2 men). The median age of onset was 60 (range 39-69). Three patients presented with central nervous system symptoms only, including confusion, seizure, stroke, and ataxia. The two others presented with systemic B-symptoms, one also with peripheral nervous system symptoms and the other also with multi-organ failure. Findings on brain imaging included white mater lesions, infarcts, hemorrhages, or combinations of these. Histology showed CD20 positive B-lymphocytes confined to small size vessels in autopsy or biopsy specimens from brain or muscle. The patient with multi-organ failure also had diffuse infiltration to spleen, liver, and kidney. Three patients died within 3 months after clinical presentation and were diagnosed at autopsy. Two others were diagnosed by biopsy, underwent chemotherapy (CHOP-R or MTX+Rituximab), and were alive one year after diagnosis.

Conclusions Although IVLBL has distinct pathological features, its clinical presentation can be protean, ranging from mild myopathy to fulminant brain or multi-organ failure. The patient's optimal chance for survival relies on early pathological diagnosis and prompt, aggressive chemotherapy.

Authors/Disclosures
Ning Zhong, MD (KP Medical Center) PRESENTER	Dr. Zhong has nothing to disclose.
Patricia Oakes, MD	An immediate family member of Dr. Oakes has received stock or an ownership interest from 3M. Dr. Oakes has received personal compensation in the range of $0-$499 for serving as a survey participant with globalhcare.
Joshua A. Sonnen	No disclosure on file
Thomas A. Berger, MD (Dept. of Neurology, Medical University of Vienna)	Prof. Berger has received personal compensation in the range of $10,000-$49,999 for serving as a speaker at scientific meetings and participant of local and international advisory boards with various companies producing and markerting treatments for multiple sclerosis (Almirall, Biogen, Biologix, Bionorica, Celgene-BMS, Merck, Novartis, Roche, Sanofi-Genzyme, TG Therapeutics, UCB).
Bruce R. Ransom, MD, PhD (University of Washington)	No disclosure on file
W. T. Longstreth, Jr., MD, FAAN (Harborview Medical Center)	The institution of Dr. Longstreth has received research support from NIH.
Maciej M. Mrugala, MD, PhD, MPH, FAAN (Mayo Clinic)	Dr. Mrugala has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Veevo Biomedicines Inc. Dr. Mrugala has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Arbor Pharmaceuticals. Dr. Mrugala has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Astra-Zeneca. Dr. Mrugala has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Servier. Dr. Mrugala has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Medscape. Dr. Mrugala has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Merck. Dr. Mrugala has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Kyiatec . The institution of Dr. Mrugala has received research support from Arbor Pharmaceuticals. Dr. Mrugala has a non-compensated relationship as a Program Director with Society for Neuro-Oncology that is relevant to AAN interests or activities.

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