Multifocal Osteosclerotic Plasmacytoma with Polyneuropathy – POEMS Syndrome
Opichalová D. 1 , Ščudla V. 1 , Vomáčka J. 2 , † Dušek J. 3 , Mačák J. 3 , Bačovský J.
1 III. interní klinika FN a LF UP, Olomouc 2 Radiologická klinika FN a LF UP, Olomouc 3 Ústav patologie FN a LF UP, Olomouc |
|
Summary:
The POEMS syndrome is a disease with a multiorgan affection, which involves in case of full
manifestation the presence of polyneuropathy, organomegaly, endocrinopathy, monoclonal gamma-
pathy and dermal changes, associated in the majority with the presence of the osteosclerotic type
of plasmacytoma. In the submitted paper the authors present an analysis of the course of the disease
in a 70-year-old man who suffered from unusually severe sensomotor neuropathy of the lower
extremities 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 and
the presence of M-protein type IgG-lambda in serum. EMG examination revealed serious neuropathy
with affection of the axon and myelin, the lymph node manifestations of angiofollicular hyperplasia
of Castelmann’s type. In the bone marrow slight plasmacytosis of clonal character (IgG-L) with
manifestations of partial medullary fibrosis and increased osteoblastic proliferation was found.
Radiography of the skeleton revealed foci of osteosclerosis in the proximal sections of the diaphysis
of 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 and
pyridinoline), serum levels of the cytoadhesive VCAM-1 molecule, IL-2 and low testosterone levels
were found. After treatment with melfalan with corticoids, subsequent actinotherapy of osteoscle-
rotic lesions and axillary lymph nodes with intensive rehabilitation marked improvement of the
condition occurred with complete restoration of muscler strength and mobility and normalization
of indicators of the haemogram, S-B2M values, prolactin, PICP, a certain decline of values of
M-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 the
presence of M-protein it is important to search for osteosclerotic lesions, the presence of POEMS
syndrome as the baseline of intensive broadly conceived treatment.
Key words:
POEMS syndrome, monoclonal gammapathy, osteosclerotic plasmacytoma, polyneu-
ropathy, angiofollicular hyperplasia of Castelmann’s type
|