POEMS syndrome with IgA lambda monoclonal gammopathy.
The various association of polyneuropathy, organomegaly, endocrinopathy, monoclonal protein and skin changes has been called the POEMS syndrome. Herein we report a case in which all the typical clinical and laboratory findings of the POEMS syndrome were present. We emphasize as the most striking features of our case the following: the similarity with scleroderma, the peripheral nerve demyelination without the presence of antibodies against nerve components, the occurrence of an IgA lambda monoclonal gammopathy and Castleman-like features at a single enlarged lymph node. To our knowledge, this is the first such case reported in Italy.
Costa
A
A
Dipartimento di Medicina Interna e Terapia Medica, (Sezione di Clinica Medica II), IRCCS Policlinico S. Matteo, Milano, Italy.
Barosi
G
G
Piccolo
G
G
Nobile-Orazio
E
E
Liberato
N L
NL
Ascari
E
E
D004700:Endocrine System Diseases / Q000276:immunology; D006801:Humans; D007070:Immunoglobulin A / Q000276:immunology*; D007146:Immunoglobulin lambda-Chains / Q000276:immunology*; D008297:Male; D008875:Middle Aged; D009422:Nervous System Diseases / Q000276:immunology*; D010265:Paraproteinemias / Q000276:immunology*; D012871:Skin Diseases / Q000276:immunology; D013577:Syndrome
D007070:Immunoglobulin A; D007146:Immunoglobulin lambda-Chains