Case Presentation: A 73-year-old woman with a history of coronary artery disease and remote cervical cancer presented to the hospital with a two-month history of progressive leg weakness and confusion. Over the course of six weeks, symptoms progressed to flaccid paralysis and loss of sensation in both legs. Magnetic resonance imaging (MRI) demonstrated enhancing hemorrhagic lesions throughout multiple vascular territories of the brain and spinal cord infarction from the T3-T11 levels. Lumbar puncture performed after steroid initiation was bland without clonal populations. Biopsy of a frontal lobe brain lesion primarily demonstrated necrotizing vasculitis and rare degenerating CD20-positive intraluminal B cells, raising concern for primary central nervous system vasculitis, systemic vasculitis or intravascular lymphoma.
The patient was transferred to a tertiary care center. On arrival she was intermittently somnolent and delirious with bilateral lower extremity paralysis and T9/10 sensory level. Admission course was complicated by several partial seizures. She was found to have anemia and thrombocytopenia. LDH was elevated to 1,095 U/L and beta-2 microglobulin to 6.1 mg/L. There was no evidence of renal or ocular vasculitis. ANA, ANCA, hepatitis and HIV serologies, and RPR were negative. Ultimately, two skin biopsies of the thigh (one of a nonspecific erythematous plaque, the other of a prominent telangiectasia) both demonstrated intravascular atypical lymphocytes with immunophenotype consistent with intravascular large B cell lymphoma.
Discussion: Intravascular large B cell lymphoma is a rare and aggressive subtype of large B cell lymphoma characterized by proliferation of neoplastic lymphoid cells in the vascular lumina of small blood vessels. Clinical manifestations vary but center upon a pathophysiology of small vessel occlusion, leading to potential misdiagnosis as small-vessel vasculitis. Patients in Western countries are more likely to present with skin involvement and neurologic symptoms, while those in Asia have higher rates of bone marrow and visceral organ involvement. Cerebrospinal fluid is often uninvolved, even if disease is primarily neurologic. Diagnosis requires tissue biopsy demonstrating intravascular lymphoid cells with characteristic B cell markers, with multiple skin biopsies recommended even if concerning skin lesions are not present.
Conclusions: The patient was started on R-CHOP. Her clinical course remains tenuous at this time. Though patients can have significant response to treatment, the presence of cord infarction portends poor prognosis for the chance of regaining lower extremity function. The rapid progression of this patient’s neurologic deficits and delay in diagnosis between indeterminate brain biopsy and diagnostic skin biopsies highlights the bewildering array of symptoms seen in intravascular lymphoma, as well as the need to consider this aggressive albeit rare condition in our clinical armamentarium.