Neurolymphomatosis associated with Sézary syndrome.
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 high-dose methotrexate sodium in treating this usually refractory complication.


METHODS
A 73-year-old woman with newly diagnosed Sézary syndrome rapidly developed severe peripheral neuropathic disease with multiple paralyses. Biopsy specimens were taken from a clinically affected nerve and the adjacent muscle; they revealed a neural infiltration by Sézary cells with secondary muscular atrophy. Partial response and major neurologic recovery occurred and persisted under high doses of intravenous methotrexate until the patient died 14 months after the Sézary syndrome diagnosis from a pericarditis of uncertain origin.


CONCLUSIONS
This unusual and demonstrative case report highlights the possible neurotropism of malignant cells in Sézary syndrome and suggests the effectiveness of high doses of intravenous methotrexate in this rare and fatal disorder.
Bezier
Maud
M
Department of Dermatology, Robert Debré Hospital, 51092 Reims Cedex 51092, France.
Reguiaï
Ziad
Z
Delaby
Philippe
P
Laroche
Liliane
L
Saïd
Gérard
G
Bernard
Philippe
P
Grange
Florent
F
D000368:Aged; D005260:Female; D006801:Humans; D009133:Muscular Atrophy / Q000209:etiology / Q000473:pathology; D010525:Peripheral Nerves / Q000473:pathology*; D012751:Sezary Syndrome / Q000188:drug therapy / Q000473:pathology*; D012878:Skin Neoplasms / Q000188:drug therapy / Q000473:pathology*