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POEMS syndrome is a rare multisystemic disorder characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin change. Although patients with POEMS are usually younger than symptomatic multiple myeloma patients, the median age of patients with POEMS syndrome is reported to be 51 years (range: 30 to 83 years). A 15-year-old boy visited our hospital because of progressive lower extremity weakness and gait disturbance. He showed polyneuropathy, hepatosplenomegaly, hypogonadism, hyperprolactinemia, adrenal insufficiency, IgG lambda type monoclonal gammopathy, hyperpigmentation, hypertrichosis, and skin thickening, which we diagnosed as POEMS syndrome. This might be the youngest case of POEMS syndrome in Korea; thus we report here with a review of literatures.(Korean J Med 70:S328-S334, 2006)
Key Words : POEMS syndrome, Age, Autologous stem cell transplantation |
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