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Síndrome de POEMS associado a doença de Castleman : a case report
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O Síndrome de POEMS é uma entidade clínica rara, de índole paraneoplásica, cujo acrónimo representa as suas principais manifestações – polineuropatia (P), organonomegália (O), endocrinopatia (E), gamapatia monoclonal (M), alterações da pele (S). A sua fisiopatologia é intrincada e carece ainda de investigação, assumindo-se no entanto que o Fator de Crescimento Endotelial Vascular (VEGF), em conjunto com os seus co-indutores, seja responsável pelas manifestações cardinais. Por se tratar de uma patologia rara e com uma vasta panóplia de sinais/sintomas que podem mimificar outras patologias mais frequentes, o seu diagnóstico é frequentemente tardio e exige um elevado nível de suspeição clínica. Estima-se que cerca de 11-30% dos doentes com Síndrome de POEMS exibam concomitantemente Doença de Castleman (DC). Dado que existe partilha de vias fisiopatológicas, principalmente ao nível da produção de IL-6 e VEGF, foi proposto um sinergismo entre ambas as patologias.
O presente Case Report descreve um quadro clássico de Síndrome de POEMS, associado a DC, sendo ilustrativo da complexidade diagnóstica e raridade destas duas patologias.
POEMS syndrome is a rare parenoplasic syndrome, whose acronym stands for the main manifestations - polyneuropathy (P), organomegaly (O), endocrinopathy (E), monoclonal gammopathy (M) and skin abnormalities (S). Its pathophysiology is intricate and still requires investigation. However, it is assumed that the Vascular Endothelial Growth Factor (VEGF), together with its co-inducers, is responsible for the cardinal manifestations. Due to its rarity and multiplicity of symptoms that mimic other more frequent pathologies, POEMS is frequently a late diagnosis, requiring high levels of clinical suspicion. It is estimated that about 11-30% of patients with POEMS syndrome, exhibit concomitant Castleman Disease (CD). Since POEMS syndrome and CD share pathophysiological features, mainly regarding VEGF and IL-6 production, a synergism is proposed between these diseases. This case report describes a classic POEMS syndrome, associated with Castleman's disease, emphasizing the rarity and diagnostic complexity of these pathologies.
POEMS syndrome is a rare parenoplasic syndrome, whose acronym stands for the main manifestations - polyneuropathy (P), organomegaly (O), endocrinopathy (E), monoclonal gammopathy (M) and skin abnormalities (S). Its pathophysiology is intricate and still requires investigation. However, it is assumed that the Vascular Endothelial Growth Factor (VEGF), together with its co-inducers, is responsible for the cardinal manifestations. Due to its rarity and multiplicity of symptoms that mimic other more frequent pathologies, POEMS is frequently a late diagnosis, requiring high levels of clinical suspicion. It is estimated that about 11-30% of patients with POEMS syndrome, exhibit concomitant Castleman Disease (CD). Since POEMS syndrome and CD share pathophysiological features, mainly regarding VEGF and IL-6 production, a synergism is proposed between these diseases. This case report describes a classic POEMS syndrome, associated with Castleman's disease, emphasizing the rarity and diagnostic complexity of these pathologies.
Descrição
Trabalho Final do Curso de Mestrado Integrado em Medicina, Faculdade de Medicina, Universidade de Lisboa, 2017
Palavras-chave
Síndrome de POEMS Doença de Castleman Polineuropatia Organomegália Endocrinopatia Gamapatia monoclonal