Multifocal Osteosclerotic Plasmacytoma with Polyneuropathy – POEMS Syndrome


Authors: D. Opíchalová 1;  V. Ščudla 1;  J. Vomáčka 2;  J. † Dušek 3;  J. Mačák 3;  J. Bačovský
Authors‘ workplace: III. interní klinika FN a LF UP, Olomouc 2 Radiologická klinika FN a LF UP, Olomouc 3 Ústav patologie FN a LF UP, Olomouc 1
Published in: Čes. Revmatol., , 2001, No. 1, p. 31-34.
Category:

Overview

The POEMS syndrome is a disease with a multiorgan affection, which involves in case of fullmanifestation the presence of polyneuropathy, organomegaly, endocrinopathy, monoclonal gamma-pathy and dermal changes, associated in the majority with the presence of the osteosclerotic typeof plasmacytoma. In the submitted paper the authors present an analysis of the course of the diseasein a 70-year-old man who suffered from unusually severe sensomotor neuropathy of the lowerextremities and who eventually developed the complete picture of POEMS syndrome, incl. hyper-pigmentation with hypertrichosis, multiple skin angiomas and bilateral axillary lymphadenomega-ly. Laboratory tests revealed, consistent with the usual picture, polyglobulia, thrombocytosis andthe presence of M-protein type IgG-lambda in serum. EMG examination revealed serious neuropathywith affection of the axon and myelin, the lymph node manifestations of angiofollicular hyperplasiaof Castelmann’s type. In the bone marrow slight plasmacytosis of clonal character (IgG-L) withmanifestations of partial medullary fibrosis and increased osteoblastic proliferation was found.Radiography of the skeleton revealed foci of osteosclerosis in the proximal sections of the diaphysisof the humerus but a normal appearance on examination of the skeleton by scintigraphy and DEXA.Raised values of FSH, prolactin, C-peptide, indicators of bone turnover (telopeptide, osteocalcin andpyridinoline), serum levels of the cytoadhesive VCAM-1 molecule, IL-2 and low testosterone levelswere found. After treatment with melfalan with corticoids, subsequent actinotherapy of osteoscle-rotic lesions and axillary lymph nodes with intensive rehabilitation marked improvement of thecondition occurred with complete restoration of muscler strength and mobility and normalizationof indicators of the haemogram, S-B2M values, prolactin, PICP, a certain decline of values ofM-protein occurred and normalization of the number of plasmocytes in bone marrow.From the submitted paper ensues that in all obscure polyneuropathies in middle age with thepresence of M-protein it is important to search for osteosclerotic lesions, the presence of POEMSsyndrome as the baseline of intensive broadly conceived treatment.

Key words:
POEMS syndrome, monoclonal gammapathy, osteosclerotic plasmacytoma, polyneu-ropathy, angiofollicular hyperplasia of Castelmann’s type

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Labels
Dermatology & STDs Paediatric rheumatology Rheumatology
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