POEMS syndrome: challenges in the diagnosis of an atypical case
DOI:
https://doi.org/10.11606/issn.1679-9836.v101i5e-196228Keywords:
POEMS syndrome, Differential diagnosis, Paraproteinemias, Paraneoplastic polyneuropathy, Diseases of endocrine systemAbstract
Introduction: POEMS syndrome is a rare paraneoplastic event, with no current report in the literature about its real prevalence. Most cases occur in middle-aged men. Case Report: Male patient, 65 years old, complaining of edema and paresthesia in the legs that evolved to plegia, associated with hyporexia and fatigue. Initial outpatient investigation revealed Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP) of undefined etiology. Excluding secondaries, the patient was diagnosed with primary hypothyroidism, severe hypogonadism, hyperchromic skin lesions, ascites, pleural effusion and thrombocytosis, in addition to monoclonal IgA Lambda gammopathy by serum immunofixation. Viral serologies was negative. Excluding the possibility of Multiple Myeloma and other gammopathies, the hypothesis of POEMS Syndrome was raised, and plasma VEGF measurement was performed (425 pg/mL; RV = <96.2). This is an atypical case in that bone lesions, present in up to 97% of cases, were not evidenced in the patient in question, making the diagnosis challenging. Conclusions: The diagnosis of rare syndromes, although challenging, brings the clinician a broader view of the patient as it increases clinical reasoning.
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Copyright (c) 2022 Rodrigo Rufino Pereira Silva, Mariana Andrade de Figueiredo Martins, Breno Antônio de Albuquerque Lobo Costa, Talina Tassi Saraiva de Arruda, Arilson Santos Alves da Silva, Yanne Almeida de Aguiar, Paulo Bernardo da Silveira Barros Filho
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