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Washington University Experience | NEOPLASMS (HEMATOLYMPHOID) | Lymphoma, Intravascular | 8A0 Case 8 History
Case 8 History ---- The patient is a 49-year-old woman with a past medical history significant for Tangier's disease and IgA hypergammaglobulinemia who presented to BJH for worsening mental status and fevers. In Jan/Feb 2015 she became progressively altered. MRI studies showed white matter lesions suspicious for ADEM. She was started on steroids and improved significantly, but not to baseline, and she was discharged on a steroid taper. After approximately four days, her mental status and motor function worsened. A repeat MRI showed new lesions. At BJH a large array of testing was mostly unrevealing. The Neuroimmunology Service thought her presentation was consistent with ADEM complicated by hemorrhage. She was restarted on steroids, underwent plasmapheresis, showed significant improvement, and was discharged on a steroid taper. However, as steroids were tapered, she began to deteriorate gradually. Over the week prior to her final presentation, she became essentially unresponsive. She had a urinary tract infection. She was started on multiple antibiotics, as well as high dose methylprednisolone. CSF cytologies, as well as a bone marrow aspirate showed no evidence of malignancy. A left vastus lateralis muscle biopsy performed showed diffuse large B-cell lymphoma, non-germinal center type. Due to the poor prognosis and, according to the patient's wishes, care was redirected and she died on 5/30 ---- At autopsy the weight of the unfixed brain was 1200g. The sulcal-gyral pattern was diffusely effaced and softened, consistent with cerebral edema. Coronal sections of the cerebral hemispheres revealed diffuse effacement of the sulci, and multifocal, patchy, white matter discoloration. Sections show widespread involvement of leptomeningeal and intraparenchymal vessels by numerous discohesive, partially necrotic, malignant-appearing cells. In addition, there are multifocal areas of subacute cerebral cortical and white matter infarction.
