POLYNEUROPATHY, ORGANOMEGALY, ENDOCRINOPATHY AND SKIN CHANGES IN A CASE OF SOLITARY MYELOMA

  • 27 September 1986
    • journal article
    • research article
    • Vol. 116  (39) , 1326-1331
Abstract
The case is reported of a 32-year-old women with polyneuropathy, organomegaly, edema, endocrinopathy, dark skin and solitary myeloma with monoclonal gammopathy of IgG/lambda type. More than 100 cases of this rare disorder have been observed up to now, mainly in Japan. In recent reviews the terms POEMS-syndrome or Crow-Fukase-syndrome have been used. Local radiotherapy of the myeloma led to lasting regression of symptoms. Studies with immunocytochemistry and immunoelectroblotting revealed specific antibody activity against hypophysis, suggesting that the pathologic monoclonal myeloma antibodies may damage the neuroendocrine centers in hypothalamus and hypophysis as the primary target. Most of the multisystemic symptoms would thus be explained as secondary alterations.