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Título

POEMS syndrome: case report

RESUMO

Case Report A 70-year-old woman was admitted to our hospital with a 2-year history of complaints of lower limb paresthesia, imbalance, ascending paresis to the upper limbs culminating in bedridden and weight loss of 30 kg. Physical examination revealed hippocratic facies, diffuse muscular atrophy, severe bilateral hand contracture, non-painful hepatomegaly and lower limb edema with acrocyanosis. Neurological examination showe distal weakness, generalized hyporeflexia and pallhypesthesia, hypoesthesia of the legs. Laboratory tests results showed a normal blood count and renal function, proteinuria and elevated TSH and VEGF. Electroneuromyography revealed sensorimotor peripheral polyneuropathy with a demylinating pattern with axonal loss and Lumbar puncture had albuminocytological dissociation. Computer tomography showed bilateral pleural effusions, ascitis and osteosclerotic lesions at the vertebral body of L3, L4, and sacroiliac. Further investigation exhibited protein electrophoresis with a distortion in gamma zone and immunofixation elevated free light chains. Bone marrow biopsy showed a reactive and hypercellular marrow with interstitial plasmocytosis and polyclonal free light chain elevation. Discussion POEMS syndrome is definided by the presence of a monoclonal plasma cell disorder, peripheral neuropathy, and one or more of the following features: osteosclerotic myeloma, Castleman disease, increased levels of serum vascular endothelial growth factor (VEGF), organomegaly, endocrinopathy, edema, typical skin changes, and papilledema. The pathogenesis of syndrome is not well understood, VEGF is the cytokine that correlates best with disease activity. Our patient exhibited most of the diagnostic criteria for POEMS. The most disabling feature of POEMS is the demyelinating neuropathy, frequently misdiagnosed as CIDP (chronic inflammatory demyelinating polyradicuneuropathy). A typical POEMS neuropathy is a symmetrical, sensorimotor, length-dependent painful neuropathy, which progresses proximally over time. Treatment is aimed at the clonal hematologic disease and the cytokine drive and supportive care for the neurological and endocrine consequences of the disease. Final Comments The aim of this study was to report a case of POEMS syndrome, emphasizing the importance of this condition, which, although rare, it should be part of the differential diagnosis of polyneuropathy.

Palavras Chave

Área

Neuropatias Periféricas