Intravascular CNS Lymphoma: An elusive diagnosis (P2-5.016)

Abstract

<h3>Objective:</h3> To report a rare case of intravascular CNS lymphoma presenting with subacute neuropsychiatric symptoms and multifocal cerebral infarcts and hemorrhages. <h3>Background:</h3> Intravascular lymphoma is a rare and aggressive subtype of extra-nodal non-Hodgkin lymphoma. It is characterized by proliferation of neoplastic B-lymphocytes within the lumen of small blood vessels, especially capillaries and post-capillary venules. Presentation is variable and depends on the organ dysfunction due to small vessel occlusion. Most common organs involved are CNS, skin and lungs. Given the rarity of the disease, variable presentation and absence of an extravascular tumor mass or peripheral blood abnormalities, the diagnosis can be extremely challenging and is often delayed. <h3>Design/Methods:</h3> NA <h3>Results:</h3> A 62-year old healthy woman presented with transient left hemiplegia and left facial numbness and was diagnosed with transient ischemic attack. In one month, she developed dysarthria and left hemibody clonic seizures without any systemic complaints. Over the next four months, she had a step-wise decline and developed severe apathy, decreased verbal fluency, diffuse hypertonia and worsening encephalopathy. Serial brain MRIs over four months showed acute-subacute infarcts and intracerebral hemorrhages involving both anterior and posterior circulation bilaterally with scattered micro-hemorrhages. Extensive work up, including CT-angiogram and PET-CT were unrevealing. CSF was notable for high protein level without pleocytosis and negative infectious and autoimmune workup. DSA was unrevealing. She did not respond to high dose steroids, intravenous immunoglobulin and cyclophosphamide. Brain biopsy revealed CNS intravascular large B-cell lymphoma. Patient was started on chemotherapy with HyperCVAD-R protocol with no improvement. <h3>Conclusions:</h3> Intravascular CNS lymphoma is a challenging diagnosis. It presents as subacute-chronic CNS vasculopathy with multifocal infarcts and hemorrhages and can mimic other CNS vasculopathies, including primary CNS angiitis and amyloid-related angiopathy. Tissue biopsy is the diagnostic gold standard and should be considered early if poor response to immunotherapy is noted. Despite high-dose chemotherapy, prognosis is poor. <b>Disclosure:</b> Dr. Ticku has nothing to disclose. Dr. Serra has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Biogen Idec. Dr. Serra has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Bristol Myers Squibb.