SESSION TYPE: Critical Care Case Report Posters
PRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PM
INTRODUCTION: Undifferentiated neurologic dysfunction represents an opportunity to expand the differential diagnosis. This case illustrates the importance of considering intravascular large B-cell lymphoma (IVLBCL) in patients with undiagnosed neurologic complaints.
CASE PRESENTATION: A 69 year old man presented to the emergency department with progressive lower extremity weakness and paresthesias. He was in good health three months prior when these symptoms began in his feet and ascended. He underwent evaluations at local hospitals that included imaging and two lumbar punctures. No etiology was determined, but he was treated with intravenous corticosteroids and initially had mild improvement in symptoms. He was sent to rehabilitation where his symptoms progressed with loss of bowel and bladder control and paraplegia. In the emergency department, his neurologic exam was significant for lower extremity paresis, areflexia, sensory deficits to the level of T6, and altered sensorium. His mental status worsened requiring transfer to the intensive care unit for mechanical ventilation. Repeat brain magnetic resonance imaging (MRI) demonstrated progressive, diffuse white matter signal abnormalities. Spine MRI did not demonstrate cord lesions. Electromyography demonstrated a mild sensory neuropathy without evidence for inflammatory demyelination. Cerebral spinal fluid was significant for protein of 100 mg/dL (15-45 mg/dL) and oligoclonal bands. Complete blood counts, liver function tests, and metabolic panels were all normal. The patient’s hospitalization was complicated by venous thromboembolism, prolonged mechanical ventilation with ventilator associated pneumonia, seizures, and worsening mental status. He was transitioned to comfort care and subsequently expired. Autopsy revealed IVLBCL in the brain, lungs, liver, adrenal glands, and bone marrow.
DISCUSSION: IVLBCL is a rare, aggressive and often fatal form of diffuse large B-cell lymphoma. It is characterized by malignant growth in small vessels of extranodal organs. Older patients are affected and present with skin lesions, non-specific neurologic complaints, anemia, or dyspnea. The vague presentation makes diagnosis difficult, and without a high index of suspicion, patients are often diagnosed post-mortem. The few published case series have reported a variety of neurological findings including dementia, tremor, seizures, paresis, sensory deficits and altered sensorium. Diagnosis can be made by biopsy of the affected organ. Anthracycline agents and rituximab have been used for the treatment of IVLBCL with improved survival; however, central nervous involvement portends a worse prognosis.
CONCLUSIONS: Unexplained neurologic symptoms should alert clinicians to consider IVLBCL as this is a rare but fatal illness, and recent treatment advances can improve survival.
1) Shimada K, et.al. Presentation and management of intravascular large B-cell lymphoma. Lancet Oncol 2009; 10: 895-902.
DISCLOSURE: The following authors have nothing to disclose: Patrick Meehan, Michael Ezzie
No Product/Research Disclosure InformationThe Wexner Medical Center at The Ohio State University, Columbus, OH