[POEMS syndrome. A rare variant of osteosclerotic multiple myeloma with polyneuropathy, organomegaly, endocrinopathy, M-gradient and skin lesions].
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.


METHODS
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.


CONCLUSIONS
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.
Cohnen
M
M
Abteilung für Hämatologie, Universität Gesamthochschule Essen.
Uppenkamp
M
M
Meusers
P
P
Brittinger
G
G
D003937:Diagnosis, Differential; D003952:Diagnostic Imaging; D006801:Humans; D007070:Immunoglobulin A / Q000097:blood; D007146:Immunoglobulin lambda-Chains / Q000097:blood; D008297:Male; D008875:Middle Aged; D016878:POEMS Syndrome / Q000175:diagnosis*; D010384:Pelvic Bones / Q000473:pathology
D007070:Immunoglobulin A; D007146:Immunoglobulin lambda-Chains
POEMS-Syndrom. Seltene Variante des osteosklerotischen multiplen Myeloms mit Polyneuropathie, Organomegalie, Endokrinopathie, M-Gradient und Hautläsionen.