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J Coll Physicians Surg Pak ; 27(10): 651-653, 2017 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-29056130

RESUMO

POEMS (acronym for polyneuropathy, organomegaly, endocrinopathy, M protein myeloma and skin changes), is a rare disease which occurs in the setting of plasma cell dyscrasias. We describe a case of an adult lady who presented with gradual onset weakness of all four limbs and multisystem involvement characterized by pedal edema, ascites, hyperpigmentation and hypogonadism. Nerve conduction study showed severe sensorimotor polyneuropathy. Serum immunofixation showed lambda light chain restricted monoclonal gammopathy. Bone marrow biopsy consistent with plasma cell dyscrasia. Hormonal assay showed decreased FSH, LH and estradiol levels which led us to diagnosis of hypogonadotrophic hypogonadism. The patient responded well to combination therapy of thalidomide, melphalan and dexamethasone. Eight months after the therapy, she noted decreased paresthesias and increased strength. She had reduced edema and ascites.


Assuntos
Hipogonadismo/diagnóstico , Hipogonadismo/etiologia , Síndrome POEMS/diagnóstico , Polineuropatias/diagnóstico , Polineuropatias/etiologia , Adulto , Dexametasona/uso terapêutico , Feminino , Humanos , Hipogonadismo/sangue , Melfalan/uso terapêutico , Síndrome POEMS/complicações , Síndrome POEMS/tratamento farmacológico , Paraproteinemias , Polineuropatias/sangue , Talidomida/uso terapêutico , Resultado do Tratamento
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