The POEMS syndrome: report of six cases.
We report six patients affected by POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, and Skin changes), a peculiar multiorgan disease frequently associated with osteosclerotic myeloma or other plasma cell disorders. Sensorimotor polyneuropathy was associated with multisystem involvement in all of the patients, with osteosclerotic myeloma in 2 cases, monoclonal gammopathy of undetermined significance in 2 cases and Castleman's disease in the final two. In all of the patients, sural nerve biopsy findings were consistent with a mixed, axonal and demyelinating neuropathy. Increased levels of Interleukin-6 were found in two cases, but the pathogenesis of the disease is far from established.
Pareyson
D
D
Istituto Nazionale Neurologico, C. Besta, IRCCS, Milano.
Marazzi
R
R
Confalonieri
P
P
Mancardi
G L
GL
Schenone
A
A
Sghirlanzoni
A
A
D000368:Aged; D001853:Bone Marrow / Q000473:pathology; D005871:Castleman Disease / Q000134:cerebrospinal fluid / Q000473:pathology; D004594:Electrophysiology; D006728:Hormones / Q000097:blood; D006801:Humans; D015850:Interleukin-6 / Q000134:cerebrospinal fluid; D008198:Lymph Nodes / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009046:Motor Neurons / Q000502:physiology; D009431:Neural Conduction / Q000502:physiology; D010026:Osteosclerosis / Q000473:pathology; D016878:POEMS Syndrome / Q000134:cerebrospinal fluid / Q000473:pathology / Q000503:physiopathology*; D010265:Paraproteinemias / Q000473:pathology; D011379:Prognosis; D013497:Sural Nerve / Q000473:pathology; D014409:Tumor Necrosis Factor-alpha / Q000134:cerebrospinal fluid
D006728:Hormones; D015850:Interleukin-6; D014409:Tumor Necrosis Factor-alpha