PERIPHERAL NERVOUS-SYSTEM INVOLVEMENT IN A PATIENT WITH LARGE T-CELL LYMPHOMA ARISING FROM A PREEXISTING MYCOSIS-FUNGOIDES

A 29-year-old man was examined. for disseminated erythematous scaling patches and plaques and reddish, partially ulcerated nodules, Histolog ical examination showed a dense, diffuse, epidermotropic infiltrate lo cated in the entire dermis to the subcutaneous tissue, composed mainly of large pleomorphic T lymphocytes, Immunohistochemistry revealed pos itivity of neoplastic cells for T-cell-associated markers, negativity for CD30 antigen and for B-cell markers. Polymerase chain reaction ana lysis detected a clonal amplification of T-cell receptor gamma. Based on clinicopathological and molecular findings, the diagnosis of large T-cell lymphoma (LCL) arising from a pre-existing mycosis fungoides wa s made. Seven months after primary diagnosis, meningeal and peripheral nervous system involvement developed with no other evidence of system ic disease. Despite chemotherapy and radiation therapy, the patient di ed 3 months after the diagnosis of nervous system involvement. In pati ents with cutaneous LCL, mild neurological symptoms may precede the co mplete diagnostic picture by some weeks. A rapid and fatal progression characterizes the clinical course of the disease.