Dados do Trabalho

Título

POEMS Syndrome: A Brazilian case series

Resumo

Introduction: Polyneuropathy, organomegaly, endocrinopathy, monoclonal-protein
and skin changes (POEMS) is a rare paraneoplastic syndrome. It may be difficult to
diagnose, specialty in developing countries facing specialist’s shortage. In this study
we sought to describe clinical, laboratory and electrophysiological characteristics
found in 19 patients diagnosed in a Brazilian referral center.

Objective to describe the clinical and laboratory characteristics of POEMS syndrome in a Brazilian terciary center

Methods POEMS diagnosis was based according to current diagnostic criteria.
Clinical data was retrospectively collected.

Results. Fourteen out of 19 patients were male (73.7%). The mean age at onset was
48,6 years old (range 38 to 66) and the mean time for diagnosis was 13,7 months
(range 5 to 48). Nerve conduction studies from all patients revealed a sensitive-
motor demyelinating neuropathy. Six patients underwent nerve biopsy, and none had
inflammatory infiltrate. Papilledema was present in 7 patients (36,8%). All cases had
albuminocytological dissociation. A lambda monoclonal component was detected in
serum from all cases, but clonal plasma cells on bone marrow biopsy were found in
only 8 cases. Bone lesions was found in 18 cases (94,7%). Sixteen (84%) had
typical skin changes. Endocrinopathy was present in 17 cases (89,9%), .
lymphadenomegaly in 6 (31,5%), splenomegaly in 9 (47%), hepatomegaly in 6
(31,5%) and thrombocytosis in 13 (68,4%), respectively. Two cases had Castleman’s disease.

Conclusion: POEMS syndrome is a rare condition. Our data suggest that the
prevalence of endocrinopathy and bone lesions in our cohort were more frequently
found than expected, and may be related to a longer time of disease duration
leading.

Palavras Chave

neuropathy POEMSsyndrome PIDC CIDP

Área

Neuropatias Periféricas