ISSN:
1590-3478
Keywords:
polyneuropathy
;
POEMS syndrome
;
monoclonal gammopathy
;
osteosclerotic myeloma
;
Castleman's disease
;
cytokines
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Description / Table of Contents:
Sommario Descriviamo 6 pazienti con sindrome POEMS (Polineuropatia, Organomegalia, Endocrinopatia, gammopatia Monoclonale, alterazioni cutanee [Skin changes]), una peculiare malattia ad interessamento multisistemico frequentemente associata a mieloma osteosclerotico o ad altre malattie plasmacellulari. Una polineuropatia sensitivo-motoria si accompagnava ad interessamento pluriviscerale in tutti i pazienti, a mieloma osteosclerotico in due casi, a gammopatia monoclonale di incerto significato in altri due, e a malattia di Castleman negli ultimi due pazienti. In tutti i 6 casi la biopsia del nervo surale dimostrava una neuropatia mista, demielinizzante ed assonale. In due casi sono stati trovati elevati livelli di Interleuchina-6, ma la patogenesi della malattia è ancora ignota.
Notes:
Abstract 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.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/BF02339932
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