POEMS Syndrome: a clinical challenge / Síndrome de POEMS: um desafio clínico

• Autor(a) principal: Brito, Vanessa de Sousa
• Data de Publicação: 2022
• Outros Autores: Freua, Fernando, Freitas, Christian Henrique de Andrade
• Tipo de documento: Artigo
• Idioma: eng
• Título da fonte: Brazilian Journal of Health Review
• Texto Completo: https://ojs.brazilianjournals.com.br/ojs/index.php/BJHR/article/view/43894
• topic: POEMS syndrome; chronic inflammatory demyelinating polyneuropathy; hypergammaglobulinemia

A 42-year-old male patient admitted to our service with a history of progressive and symmetrical weakness, paresthesia of the lower limbs, weight loss and urinary retention for about 8 months. Physical examination demonstrated poor general condition, lower limb edema, hepatomegaly and digital clubbing. Neurological examination showed a polyneuropathic pattern, with hypoesthesia and absence of deep reflexes of the lower limbs. Initial laboratory investigation demonstrated erythrocytosis and thyroid and adrenal dysfunction in addition to hypergammaglobulinemia. CSF analysis, due to the clinical suspicion of chronic inflammatory demyelinating polyneuropathy, resulted in protein-cytological dissociation. PIDC was hypothesized to be due to light-chain disease, but for this association we supplemented with bone marrow analysis and whole-body CT scan, which showed 4% atypia in plasma cells and osteolytic and osteoblastic lesions in the vertebro-costal region between the 8th and 9th vertebrae. Subsequent biopsy confirmed plasmacytoma. He performed autologous bone marrow transplantation, but evolved with infectious complications and died within 3 months.