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Washington University Experience | NEOPLASMS (HEMATOLYMPHOID) | Lymphoma, Intravascular | 13A0 Case 13 History
Case 13 History ---- The patient was a 60-year-old woman with a history of hypertension, hyperlipidemia, diabetes mellitus and sickle cell trait, who presented with a steroid-responsive encephalopathy and stroke syndrome, thought to be lupus cerebritis. In January 2013 she had multiple left MCA distribution subcortical strokes thought to reflect left internal carotid artery stenosis; several similar infarcts resulted in readmission in early 10/2014 and 12/2014. Multiple CSF analyses, skin and muscle biopsy, were negative for malignancy but clinically there was concern for intravascular lymphoma. Marked improvement of mental state followed high-dose intravenous steroids and the patient was discharged. Word-finding difficulty and bilateral lower extremity weakness developed with MRI evidence of multiple punctate infarcts. Again, extensive laboratory work-up revealed no infectious etiologies and a bone marrow aspirate was likewise negative for malignancy. High-dose intravenous steroids and a dose of Cytoxan improved mental status. Worsening clinical course suggested new strokes and she developed seizures. MR imaging showed extensive microangiopathic disease throughout the cerebral hemispheres bilaterally. An EEG showed seizure activity which was not improved with drugs. Steroids were held until a brain biopsy could be obtained. The day of the planned biopsy, the patient had worsening mental status with blown pupils and CT evidence of uncal and tonsillar herniation which did not respond to 1000 mg of methyl-prednisolone; osmotic agents were withdrawn due to elevated sodium levels. A brain death exam was performed and the patient was declared deceased. ---- At autopsy the weight of the unfixed brain was 1460g. Coronal sections showed diffuse effacement of the cerebral sulci, loss of gray-white matter differentiation, and multiple infarcts involving both gray and white matter, bilateral basal ganglia and thalami. The white matter throughout appeared friable, especially in the corpus callosum. Radial sections of the cerebellum showed hemorrhage and softening of the bilateral tonsils. ---- There is widespread involvement of leptomeningeal, and intraparenchymal vessels by numerous intravascular discohesive, partially necrotic, malignant-appearing cells, especially the occipital lobe. The intravascular tumor cells are immunopositive for CD3, CD56, and granzyme as well as for in-situ hybridization of the EBV virus (EBER). Rare, small cells are immunopositive for CD20. All tumor cells are immunonegative for CD30, Alk-1, and EMA. A Ki-67 immunostain labels a subset of tumor cells. Overall, the lymphoma immunophenotype best fits with the designation of an NK/T-cell lymphoma. Additional pertinent findings include negative ante-mortem skin and bone marrow biopsies. This particular case is rather unusual because most intravascular lymphomas are of B-cell immunophenotype with only rare case reports of NK/T cell intravascular lymphomas with CNS involvement. Such cases, similar to ours, are documented to be EBER positive.
