Intravascular Large B-Cell Lymphoma Presenting as Migraine with Aura: A Case Report

Intravascular large B-cell lymphoma is a rare, fatal, aggressive lymphoma that is characterized by the proliferation of clonal lymphocytes within the lumen of small, medium, and large vessels. Diagnosis is challenging given the nonspecific initial presentation. Incidence is <1 case per 1 million per year worldwide.

We report a case of intravascular large B-cell lymphoma in a 62-year-old female who presented to the clinic with symptoms of migraine with aura. MRI brain showed T2 hyperintensities in the splenium of the corpus callosum, right frontal, and bilateral parietal lobes, which progressed on repeat imaging. Laboratory studies only showed mild elevation of alanine aminotransferase to 75 (8-35 U/L). Computed tomography of the chest, abdomen, and pelvis showed splenomegaly but did not show lymphadenopathy or masses. She underwent a brain biopsy and was found to have intravascular large B-cell lymphoma and was transferred to a specialist oncological center to be started on R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) chemotherapy. She passed away 7 months after the initial outpatient visit.

The initial presentation of migraine with aura was not previously described. Nonspecific initial laboratory studies, preexisting Waldenstrom's macroglobulinemia which explained splenomegaly, absence of masses in the chest, abdomen, and pelvis, absence of lymphadenopathy, and MRI imaging mimicking multiple sclerosis made the diagnostic process exceedingly difficult. We demonstrate another presentation of this rare and fatal disease and hope that the reader will consider this differential diagnostic possibility when evaluating patients with rapidly worsening neurological symptoms.