P11.09A NEUROLOGIC PRESENTATION OF INTRAVASCULAR LARGE B-CELL LYMPHOMA

INTRODUCTION: Intravascular large B-cell lymphoma is a rare and fatal disease that can have central nervous system manifestations. Given its rarity and the multiplicity of presentations, diagnosis is extremely difficult. We report a case that presented with neurological manifestations and which diagnosis was made ante mortem. CASE REPORT: A 74-year-old man, with a background of hypertension and dyslipidemia was referred to our neurology department with a history of speech difficulties and walking disturbances that rapidly evolved to an acute confusional sate. Physical examination disclosed an obvious executive dysfunction, global dysphasia and gait dyspraxia. Computed tomography scan of the brain showed a right frontal paramedian subcortical hiperdensity. Further characterization with brain MRI showed that this lesion was not expansive and did not enhanced with gadolinium. On laboratory analysis there was a mild lymphopenia and increased lactate dehydrogenase and ferritin levels; erythrocyte sedimentation rate was in the normal range and the immunological and virological tests were negative. Glucocorticoids (methylprednisolone 1g, intravenous, per day, during five days) were administered, leading to some improvement in the patient's symptoms. However, the control brain MRI disclosed lesion worsening, with bihemispheric extension. The cerebrospinal fluid study was unremarkable and the anti-neuronal antibodies were negative. Control laboratory analysis revealed an increase in serum C-reactive protein, erythrocyte sedimentation rate, lactate dehydrogenase (>900 IU/l), hepatic enzymes and B2-microglobulin levels. Electroencephalography was unremarkable and there was no evidence of emboligenous cardiopathy on the transesophageal echocardiogram. A computed tomography scan of the chest abdomen and pelvis was performed, with evidence of nodular thickening of both adrenal glands. An adrenal gland biopsy was undertaken, and the histological exam confirm the diagnosis of intravascular large B-cell lymphoma. Patient's condition rapidly worsened, evolving to depression of consciousness, cortical blindness, tetraparesis with brachial diplegia, and the patient died. CONCLUSION: The clinically heterogeneous spectrum of presentation and the lack of pathognomonic features or markers have made ante mortem clinical diagnosis of intravascular large B-cell lymphoma difficult and challenging. This report highlights the diagnostic challenge, the aggressive behavior and the devastating clinical course associated with this clinical entity.