Neurolymphomatosis Associated With Sézary Syndrome

Abstract

Background: Mycosis fungoides and Sézary syndrome are cutaneous T-cell lymphomas characterized by the epidermotropism of tumor cells. Neuropathic disease is rare during mycosis fungoides and Sézary syndrome and usually results from a central nervous system involvement in late stages. Neurolymphomatosis is defined as the infiltration of the peripheral nerves by tumor lymphocytes. It has been described in patients with aggressive systemic lymphomas but, to our knowledge, not in patients with mycosis fungoides or Sézary syndrome. We report the first case of neurolymphomatosis in a patient with Sézary syndrome and the partial efficacy of highdose methotrexate sodium in treating this usually refractory complication.

Neurolymphomatosis in primary cutaneous CD4+ pleomorphic small/medium-sized T-cell lymphoma mimicking Hansen's disease

“…[234] It typically presents as any of the four clinical scenarios: (1) painful or painless peripheral mononeuropathy or mononeuritis multiplex, (2) painful peripheral polyneuropathy or polyradiculopathy, (3) painless polyneuropathy, and (4) painful or painless cranial neuropathy. [1] NL is further classified as primary and secondary.…”

“…This is due to the limited information that is currently available, delay in establishing diagnosis and inability of chemotherapeutic agents to penetrate through blood-nerve barrier into peripheral nervous system. [3]…”

Peripheral Neuropathy in Sézary Syndrome: Coincidence or a Part of the Syndrome?

“…Another case of SS with neurolymphomatosis was reported by Bezier et al [3]. In that case, muscle and nerve biopsy specimens showed neurogenic muscle atrophy and an axonal neuropathy secondary to an epineurial and endoneurial infiltration by Sézary cells [3].…”

“…Another case of SS with neurolymphomatosis was reported by Bezier et al [3]. In that case, muscle and nerve biopsy specimens showed neurogenic muscle atrophy and an axonal neuropathy secondary to an epineurial and endoneurial infiltration by Sézary cells [3]. Although extracutaneous spread is not uncommon in advanced stages of SS, neurological complications are rare and result from leptomeningeal or central nervous system involvement.…”

Neurological Complications of Non-Hodgkin Lymphoma

“…Neurolymphomatosis (reviewed in Chapter 12) causes a progressive peripheral neuropathy, secondary to lymphomatous infiltration of nerve roots and peripheral nerves [64]. PET imaging may guide selection of a nerve for biopsy, which is required for diagnosis (Fig.…”