POEMS-SYNDROME

Background: A rare multi-organ involvement in plasma-cell dyscrasias h as been named POEMS-syndrome: it is a synopsis of monoclonal gammopath y (M-gradient), osteosclerotic bone lesions. peripheral polyneuropathy , organomegaly, endocrinopathy and skin lesions. Case Report: A patien t 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 hy pergonadotropic hypogonadism, hyperprolactinaemia, and sclerotic bone lesions. In addition, he showed a changing organomegaly, and hyperpigm entation of the skin. Conclusion: As yet, aetiology and pathophysiolog y are not fully understood. Irradiation or surgical resection of one o r several osteosclerotic bone lesions may prove the polyneuropathy or may even lead to a complete remission of all symptoms. Thus, monoclona l immunoglobulins should be searched for in any unclear polyneuropathy , as should be for other symptoms of the POEMS-syndrome.