Acute arterial obliteration: a new feature of the POEMS syndrome?
The POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammapathy, and skin changes) syndrome is a rare variant of plasma cell dyscrasia with multisystemic manifestations. We present 4 cases with arterial symptoms typical of acute arterial obliteration (AAO) and review 9 similar cases in the literature. The clinical course of AAO was unusual and particularly severe when affecting the lower limbs; recurrent events required amputations. As demonstrated by angiographic and histologic studies, thrombotic and atheromatous lesions were the main pathologic features of AAO. Atherosclerotic risk factors were absent or moderate in 3 of our cases, and no cause of thrombosis other than the POEMS syndrome was found. A high production of cytokines was found in all cases, with elevated serum levels of interleukin-1 beta (9/9 samples), interleukin-6 (7/9 samples), and tumor necrosis factor-alpha (6/9 samples). We suggest that arterial manifestations should be added to the spectrum of manifestations of the POEMS syndrome. Cytokines may mediate the POEMS syndrome-associated AAO, as previously proposed for the other systemic manifestations of this disorder.
Lesprit
P
P
Service de Médecine Interne, Hôpital Henri Mondor, Créteil, France.
Authier
F J
FJ
Gherardi
R
R
Bélec
L
L
Paris
D
D
Melliere
D
D
Schaeffer
A
A
Godeau
B
B
D000208:Acute Disease; D000368:Aged; D001162:Arteriosclerosis Obliterans / Q000209:etiology*; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D006801:Humans; D015592:Immunoradiometric Assay; D007375:Interleukin-1 / Q000097:blood; D015850:Interleukin-6 / Q000097:blood; D008297:Male; D008875:Middle Aged; D016878:POEMS Syndrome / Q000097:blood / Q000150:complications*; D014409:Tumor Necrosis Factor-alpha / Q000378:metabolism
D007375:Interleukin-1; D015850:Interleukin-6; D014409:Tumor Necrosis Factor-alpha