Síndrome de POEMS

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Abstract

El síndrome de POEMS es un trastorno paraneoplásico asociado a discrasia de células plasmáticas que reúne, entre sus criterios diagnósticos mayores, la presencia de polineuropatía y de gammapatía monoclonal, entre otras como esclerosis ósea y/o cutánea. Se reportó un caso que reúne las características dominantes del síndrome.

Translated abstract

POEMS syndrome is a paraneoplastic disorder associated with plasma cell dyscrasia which joins among itsmajor diagnostic criteria, the presence of polyneuropathy and monoclonal gammopathy, such as bone and / or skin sclerosis. a case that meets the key features of the syndrome is reported.

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Most cited references 13

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POEMS syndrome.

Angela Dispenzieri (2007)

POEMS syndrome is a rare paraneoplastic syndrome secondary to a plasma cell dyscrasia. Recognition of the complex of a combination of peripheral neuropathy, organomegaly, endocrinopathy, monoclonal plasmaproliferative disorder, skin changes, papilledema, extravascular volume overload (peripheral edema, pleural effusions, ascites), sclerotic bone lesions, thrombocytosis, Castleman disease is the first step in effectively managing the disease. A rise in the blood levels of vascular endothelial growth factor is usually confirmatory. More than 95% of patients will have monoclonal lambda sclerotic plasmacytoma(s) or bone marrow infiltration. In patients with a dominant sclerotic plasmacytoma, first line therapy should include radiation to the lesion. Retrospective analysis and personal experience would dictate that systemic therapy be considered for patients with diffuse sclerotic lesions or absence of any bone lesion and for those who have not demonstrated stabilization of their disease 3 to 6 months after completing radiation therapy. For those patients with diffuse disease, systemic therapy is indicated. Useful approaches include therapy with corticosteroids, low dose alkylator therapy, and high dose chemotherapy with peripheral blood stem cell transplant. Until the pathogenesis is fully understood, these are the mainstays of treatment for patients with POEMS syndrome. The role of anti-VEGF therapies, immune modulatory drugs, and proteasome inhibitors has not yet been defined, but drugs with known high rates of treatment related neuropathy should not be considered as first line therapy.

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Endocrinopathy in POEMS syndrome: the Mayo Clinic experience.

Gunjan Gandhi, Rita Basu, Angela Dispenzieri … (2007)

To determine the prevalence and characteristics of endocrinopathies at diagnosis of POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes) syndrome.