Unraveling the Diagnosis and Therapeutic Triumph in Intravascular CNS Lymphoma: A Case Report
Kajol Doshi1, Shravan Sivakumar1
1Department of Neurology, University of Massachusetts Chan Medical School
Background:
Intravascular lymphoma (IVL) represents a rare variant of extra-nodal non-Hodgkin's lymphoma, characterized by the confinement of lymphoma cells within the lumina of small blood vessels. This condition has a marked predilection for CNS involvement (CNS-IVL), accounting for 48% of all reported cases. CNS-IVL typically presents with non-specific symptoms and radiological findings of non-specific white matter disease and infarcts, posing significant diagnostic challenges, often leading to misdiagnoses such as CNS vasculitis and progressive multifocal leukoencephalopathy. Consequently, approximately 60% of CNS-IVL cases are diagnosed post-mortem, and the prognosis is poor, with overall survival averaging 240 days and a 3-year survival rate of only 4%.
Design/Methods:
We present a case report of CNS-IVL with a favorable neurological outcome.
Results:
A 64-year-old male with a past medical history of anemia and thrombocytopenia presented with insidious, progressive neurological symptoms over several months, including dizziness and word-finding difficulties, which subsequently evolved into ataxia, weakness, and cognitive decline. CSF analysis revealed elevated proteins with no cell counts, and flow cytometry was unremarkable. Radiological assessments with MRI of the brain and diagnostic cerebral angiogram raised concerns about the possibility of CNS vasculitis, prompting an early brain biopsy that revealed large B-Cell lymphoma in an intravascular distribution. Subsequently, the patient underwent one cycle of methotrexate and six cycles of R-CHOP therapy, resulting in remarkable improvements in executive cognitive function, ambulation, and the achievement of complete remission. As of this report, at two years post-symptom onset and one and a half years post-treatment initiation, the patient successfully returned to full-time employment, with only mild residual imbalance primarily attributed to lower extremity weakness.
Conclusions:
This case highlights the importance of obtaining an early brain biopsy in patients presenting with undifferentiated white matter disease, as timely intervention can significantly impact the course of CNS-IVL, preventing neurological deterioration and death.