Fatal peripheral neurolymphomatosis after remission of histiocytic lymphoma

A 32-year-old woman with histiocytic lymphoma was in complete clinical remission after two courses of chemotherapy, when peripheral neuropathy developed fulminantly. Abnormalities included facial nerve paralysis, dysphagia, quadriparesis, myalgia, and incontinence. She died 10 days after onset of these symptoms. Postmortem examination revealed infiltration of peripheral nerves by lymphomatous cells with no involvement of meninges, brain, lymph nodes, or other organs. Differences in the blood-brain barrier of peripheral and central nervous system are suggested: The peripheral barrier may be more penetrable by malignant histiocytes or less permeable to cytotoxic drugs. Intrathecal chemotherapeutic drug instillation and irradiation may be beneficial.