POEMS syndrome: clinical, pathological and immunological study of a case.
A young Italian woman with a POEMS syndrome is described. The patient had a plasma cell dyscrasia without clinical or laboratory evidence of multiple myeloma. The phenotypic analysis of bone marrow cells and peripheral blood lymphocytes revealed a normal pattern. The immunological study of CSF showed high levels of interleukin-6, whereas this cytokine was not detectable in the serum. Electrophysiological studies and sural nerve biopsy showed a mixed, demyelinating-axonal sensorimotor neuropathy with marked loss of large myelinated fibres. Long-term treatment with prednisone gave some clinical improvement.
Orefice
G
G
Department of Neurology, Federico II University.
Morra
V B
VB
De Michele
G
G
Fusco
C
C
Borgia
G
G
Cerini
R
R
Barbieri
F
F
D000328:Adult; D015703:Antigens, CD / Q000032:analysis; D001706:Biopsy; D001853:Bone Marrow / Q000473:pathology; D003937:Diagnosis, Differential; D004594:Electrophysiology / Q000379:methods; D005260:Female; D006801:Humans; D015850:Interleukin-6 / Q000134:cerebrospinal fluid; D008214:Lymphocytes / Q000276:immunology; D008475:Median Nerve / Q000503:physiopathology; D009046:Motor Neurons / Q000502:physiology; D009413:Nerve Fibers, Myelinated / Q000473:pathology; D009431:Neural Conduction; D009475:Neurons, Afferent / Q000502:physiology; D016878:POEMS Syndrome / Q000276:immunology / Q000473:pathology / Q000503:physiopathology*; D013497:Sural Nerve / Q000473:pathology / Q000503:physiopathology
D015703:Antigens, CD; D015850:Interleukin-6