[Polyneuropathy, organomegaly, endocrinopathy and skin changes in a case of solitary myeloma].
The case is reported of a 32-year-old woman 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 neuroendocrinic centers in hypothalamus and hypophysis as the primary target. Most of the multisystemic symptoms would thus be explained as secondary alterations.
D000328:Adult; D004700:Endocrine System Diseases / Q000175:diagnosis / Q000209:etiology* / Q000473:pathology; D005260:Female; D006529:Hepatomegaly / Q000175:diagnosis / Q000209:etiology* / Q000473:pathology; D006801:Humans; D007146:Immunoglobulin lambda-Chains; D008998:Monoclonal Gammopathy of Undetermined Significance / Q000175:diagnosis / Q000473:pathology; D009422:Nervous System Diseases / Q000175:diagnosis / Q000209:etiology* / Q000473:pathology; D010859:Pigmentation Disorders / Q000175:diagnosis / Q000209:etiology* / Q000473:pathology; D010954:Plasmacytoma / Q000150:complications* / Q000175:diagnosis / Q000473:pathology; D013163:Splenomegaly / Q000175:diagnosis / Q000209:etiology* / Q000473:pathology
D007146:Immunoglobulin lambda-Chains
Polyneuropathie, Organomegalie, Endokrinopathie und Hautveränderungen bei einem Fall mit solitärem Myelom.