Meningeal large granular lymphocyte lymphoma

A 57-year-old man who presented with leptomeningeal lymphoma was reported. The lymphoma cells in the cere-brospinal fluid were large in size, had atypical nuclei and contained many azurophilic granules in their pale cytoplasm, all of which were consistent with malignant lymphoma of large granular lymphocytes (LGL). Immunohistochemically, tumor cells were positive for cyto-plasmic CD3, UCHL-1, and CD56, markers of natural killer (NK)cells. Epstein-Barr virus encoded small RNA (EBER) was detected in tumor cells by in situ hybridization. No evidence of parenchymal central nervous system or systemic tumor was identified, although at autopsy microscopic lympuoma involvement was found in the pituitary gland and kidney. To our knowledge this is the first autopsy case report of primary meningeal LGL lymphoma.