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Washington University Experience | NEOPLASMS (HEMATOLYMPHOID) | Lymphoma, Intravascular | 4A0 Case 4 History
Case 4 History ---- The patient was a 77 year old woman with a past medical history significant for hypertension and hyperlipidemia. She presented to an OSH in November of 2011 with numbness of the right lower extremity which progressed to bilateral lower extremity weakness. In December 2011 suspected thoracic myelitis responded to IV steroids. She subsequently had two more steroid responsive relapses. In April of 2012, her symptoms ceased to improve with steroids or Azathioprine. Further workup at that time showed a normal CSF including a normal IgG index, negative NMO antibody, and positive Lyme IgM antibody. MRI showed T1-T3 and T5-T12 hyperintensities. Repeat MRI at BJH showed multiple diffuse restriction areas in the brain consistent with subacute infarcts and T10-L1 hyperintensity in spinal cord thought concerning for a demyelinating disease, infectious process or vasculitis. Serum ANA, ANCA, B12, RPR, anti-TPO and anti-TG were all normal/negative. An EMG was compatible with multi-radiculopathy versus plexopathy. The clinical team decided to empirically treat her for a possible antibody-negative NMO with plasma exchange. She underwent 1.5 sessions in April 2012, but during her second session she had chest pain with atrial fibrillation and hypotension. Tests showed decreased hemoglobin (7.7) and platelets (78K). She was also found to have paraproteinemia with increased serum gamma peak and beta 2 protein. The patient was evaluated by hematology who found circulating plasmacytoid lymphocytes and were planning for a bone marrow biopsy. Her white blood cell count increased from 8200 on 4/27 to 40,000 on 4/30. On 4/28 the patient developed right upper quadrant pain which showed increased anasarca and cholelithiasis without evidence of cholecystitis. She had increasing oxygen requirement while admitted. On return to the floor after her abdominal CT, the patient was found to be less responsive, hypotensive to the 70s systolic and with increased oxygen requirement. On 4/30 the patient was found to be hypotensive with maximum pressors and agonal breathing. At this time her husband redirected her care to comfort care only and the patient died. ---- At autopsy the weight of the unfixed brain was 1300g. The medulla and spinal cord was slightly discolored and granular, particularly in its anterior aspect. Transverse sections of the brainstem a cruciform discolored area involving the middle of the pons was noted suspicious for central pontine myelinolysis whose images are shown in the white matter section, CPM, case #6.
