[A man with plasma cell dyscrasia and polyneuropathy due to POEMS syndrome].
In a 52-year-old man with general malaise, muscle stiffness and weakness, POEMS-syndrome was diagnosed based on polyneuropathy, splenomegaly, lymphadenopathy, subclinical hypothyroidism and the presence of a monoclonal paraprotein with osteosclerotic lesions and an indurated skin (POEMS is an acronym for Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein, Skin changes). This is a rare systemic disease from the clinical spectrum of plasma cell dyscrasias with polyneuropathy. The clinical picture is broader and more pleomorphic than the acronym suggests. The possibility of a POEMS syndrome should be considered in the differential diagnosis of polyneuropathy in association with monoclonal gammopathy. Quite often it is associated with osteosclerotic myeloma or mixed osteoscleroticlytic lesions. The patient described was treated with high dose corticosteroids which were gradually decreased over the next three months, upon which a marked improvement could be seen. The general malaise subsided, as did the splenomegaly, and the skin became supple again.
Driessens
S K
SK
Universitair Ziekenhuis Leuven, Herestraat 49, B-3000 Leuven, Dienst Inwendige Geneeskunde, afd. Hematologie.
Wildiers
H
H
Verhoef
G E G
GE
Vanstraelen
D
D
Robberecht
W
W
Vandenberghe
P
P
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D003937:Diagnosis, Differential; D006801:Humans; D008297:Male; D008875:Middle Aged; D016878:POEMS Syndrome / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology; D010265:Paraproteinemias / Q000209:etiology*; D011115:Polyneuropathies / Q000209:etiology*; D012867:Skin / Q000473:pathology; D013163:Splenomegaly / Q000209:etiology
D000305:Adrenal Cortex Hormones