Abstract

A 78-year-old man sought care for saddle anesthesia, left lower extremity numbness, and bilateral lower extremity weakness. The sensory loss occurred suddenly, starting initially in the left perianal region and over the course of 3 hours extending down to involve the entirety of the left lower extremity. Symptoms were stable until 3 weeks later, when he had a few episodes of urinary incontinence. Diffuse, severe, bilateral, lower extremity weakness developed. The patient reported 6 months of intermittent night sweats. Serum studies were notable for pancytopenia and increased erythrocyte sedimentation rate and levels of ferritin and lactate dehydrogenase. Lumbar puncture showed a mildly increased protein concentration with normal blood cell count, glucose value, and cytologic and flow cytometry findings. Magnetic resonance imaging showed multifocal regions of increased T2 signal throughout the central nervous system including the cerebrum, cerebellum, upper cervical cord, lower thoracic cord, and conus medullaris. Gadolinium enhancement was present in the corpus callosum, cerebellum, and dorsal lower thoracic cord. One week later, 18F-fludeoxyglucose–positron emission tomography/computed tomography showed patchy 18F-fludeoxyglucose activity in the cerebral parenchyma, as well as 2 cutaneous, 18F-fludeoxyglucose-avid soft-tissue nodules. Fine-needle aspiration of 1 of these nodules indicated diffuse large B-cell lymphoma, with no dysplastic abnormalities identified on subsequent bone marrow biopsy. Incisional biopsy of the second soft-tissue nodule showed foci of diffuse large B-cell lymphoma adherent to the lumina of a few small arteries, consistent with a diagnosis of intravascular lymphoma. The patient was diagnosed with intravascular large B-cell lymphoma. At initial evaluation at an outside facility, empiric intravenous corticosteroids were administered. After the biopsy findings of intravascular large B-cell lymphoma, he was started on intermediate-dose methotrexate followed by rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone therapy. He continued to experience severe, bilateral, lower extremity weakness and sensory loss. Two months after diagnosis of intravascular large B-cell lymphoma, he died of medical complications from chemotherapy. Intravascular lymphoma is a rare lymphoma subtype that is typically of B-cell origin. The neoplastic cells preferentially grow within the lumen of blood vessels, potentially due to a lack of cellular machinery required for cellular extravasation and parenchymal invasion.

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