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Abstract Details

Intrasvascular B-cell lymphoma presenting as Cortical Venous Thrombosis
Neuro-oncology
Neuro-oncology Posters (7:00 AM-5:00 PM)
028
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Intravascular B-cell lymphoma (IVBCL) is a rare hematological neoplasm presenting with diverse neurological symptoms including ischemic infarcts, hemorrhage and peripheral nervous system infiltration. Here, we present a case of IVBCL presenting as multifocal cortical venous thrombosis. 
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59-year-old right-handed Guatemalan immigrant female presented with subacute onset of altered mental status, lethargy, dysarthria, transient left face and arm numbness, unintentional weight loss and poor appetite. Physical exam was remarkable for disorientation to time and place and absence of any focal neurological deficits. She was found to be pancytopenic with normal electrolytes, liver function and kidney function tests. CT head revealed two areas of hemorrhages and punctate calcifications. Magnetic resonance (MR) imaging (MRI) of brain showed edema surrounding hemorrhage. With suspicion for venous thrombosis, MR Venogram was obtained that revealed multifocal cortical vein thrombosis. MR Angiogram with blackblood imaging showed vessel wall enhancement involving peripheral arteries and veins. Cerebrospinal fluid (CSF) studies revealed slightly elevated protein and normal cell count. Malignancy screen was negative. Extensive infectious and inflammatory workup revealed latent syphilis. She developed bilateral lower extremity weakness with upgoing toes after 10 days of admission. MR Thoracic spine revealed T2 hyperintensities at the level of T10. Bone marrow biopsy revealed large atypical B-cells in the lumina of sinusoids and skin biopsy demonstrated evidence of intravascular diffuse large B-cell lymphoma although peripheral smear was negative. She eventually completed several rounds of chemotherapy and currently ambulates with a walker. 
IVBCL is a rare extra-nodal diffuse large B-cell lymphoma subtype characterized by selective growth of B-cells within lumina of small vessels due to lack of CD29 that is critical for extravasation of lymphocytes. Neurological presentations are diverse and diagnosis could be delayed given normal peripheral smear. One needs to maintain a high index of suspicion presenting with B-symptoms and ischemic or hemorrhagic strokes. 
Authors/Disclosures
Praveen Hariharan, MD (University of Minnesota)
PRESENTER
Dr. Hariharan has nothing to disclose.
No disclosure on file
No disclosure on file
No disclosure on file
No disclosure on file
Marco A. Gonzalez, MD, FAAN (Cleveland Clinic Foundation) Dr. Gonzalez Castellon has nothing to disclose.