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Washington University Experience | NEOPLASMS (HEMATOLYMPHOID) | Lymphoma, Intravascular | 6A0 Case 6 History
Case 6 History ---- The patient is a 78-year-old man with a rapidly progressive dementia and a past medical history significant for hypertension and hyperlipidemia. Prior to presentation, he had 2-3 months of fatigue and a 10-15 pound unintentional weight loss. He had one month of bilateral upper extremity tremors (thought to be essential tremor that responded to propranolol), difficulty with speech and word finding, possible visual hallucination, and personality changes. His symptoms progressed, including worsening language difficulty and changes in personality and behavior. He was admitted to BJH on 9/3 for this rapidly progressive altered mental status with worsening tremor, new lower extremity weakness, and word-finding difficulty. During that hospitalization he was found to have culture negative endocarditis (after antibiotic treatment) with vegetations, left total internal carotid artery occlusion and >50% stenosis of the right internal carotid artery (9/4). MRI was interpreted as a subacute infarct in the right postcentral gyrus and subacute infarct in the left centrum semiovale. On 10/12 he was re-admitted to BJH where brain MRI demonstrated interval acute on subacute infarcts in the bilateral centrum semiovale, consistent with watershed infarct versus embolic infarcts. He had continued fluctuating altered mental status during this time. An extensive workup for an infectious etiology causing his encephalopathy was negative. A possibility of Creutzfeldt-Jakob disease (CJD) was considered but the neurologists thought CJD was unlikely given the clinical course with two separate improvements of cognition, the lack of expected MRI findings, and a CSF tau level of 153 picograms/ml. Instead they thought that a vasculopathy was more likely. After this evaluation, the patient's clinical course steadily declined. He became hypotensive with desaturations and was transferred to the ICU on 11/17 and required cardiopulmonary resuscitation that lasted approximately 13 minutes. At this time, a urine culture grew Pseudomonas aeruginosa, and he was thought to be hypotensive from sepsis. A head computed tomography CT) showed cerebral edema. While in the ICU, he had progressive loss of brainstem function and was re-directed to comfort care and died on 11/18. ---- The weight of the unfixed brain is 1420 g (normal: 1250-1400 g). The brain was processed using the BJH CJD protocol and material frozen and fixed for National Prion Disease Surveillance Center (NPDSC). After receiving NPDSC results showing no evidence of prion disease, the fixed left hemi brain was processed for further histologic work up. ---- H&E stained sections from all areas of the brain sampled showed the presence of malignant appearing cells within small to large vessels in the brain parenchyma, in the leptomeninges, or both. The tumor cells appear confined to the intravascular spaces.
