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.
POEMS syndrome with IgA lambda monoclonal gammopathy / A. Costa, G. Barosi, G. Piccolo, E. Nobile-Orazio, N. L. Liberato, E. Ascari. - In: HAEMATOLOGICA. - ISSN 0390-6078. - 75:2(1990), pp. 170-2-172.
POEMS syndrome with IgA lambda monoclonal gammopathy
E. Nobile-Orazio;
1990
Abstract
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.Pubblicazioni consigliate
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