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  • Title: [POEMS syndrome. A rare variant of osteosclerotic multiple myeloma with polyneuropathy, organomegaly, endocrinopathy, M-gradient and skin lesions].
    Author: Cohnen M, Uppenkamp M, Meusers P, Brittinger G.
    Journal: Med Klin (Munich); 1998 Nov 15; 93(11):678-82. PubMed ID: 9872045.
    Abstract:
    BACKGROUND: A rare multi-organ involvement in plasma-cell dyscrasias has been named POEMS-syndrome: it is a synopsis of monoclonal gammopathy (M-gradient), osteosclerotic bone lesions, peripheral polyneuropathy, organomegaly, endocrinopathy and skin lesions. CASE REPORT: A patient is presented who had a classical manifestation of this disease known mainly in Japan. A monoclonal IgA-lambda-gammopathy was determined as cause of a gradually progressive polyneuropathy. The patient had a hypergonadotropic hypogonadism, hyperprolactinaemia, and sclerotic bone lesions. In addition, he showed a changing organomegaly, and hyperpigmentation of the skin. CONCLUSION: As yet, aetiology and pathophysiology are not fully understood. Irradiation or surgical resection of one or several osteosclerotic bone lesions may improve the polyneuropathy or may even lead to a complete remission of all symptoms. Thus, monoclonal immunoglobulins should be searched for in any unclear polyneuropathy, as should be for other symptoms of the POEMS-syndrome.
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