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.

[1]  F. Authier,et al.  Overproduction of proinflammatory cytokines imbalanced by their antagonists in POEMS syndrome. , 1996, Blood.

[2]  J. Suc,et al.  Renal involvement in POEMS syndrome. , 1995, Clinical nephrology.

[3]  J. Dubost,et al.  POEMS syndrome: a study of 25 cases and a review of the literature. French Study Group on POEMS Syndrome. , 1994, The American journal of medicine.

[4]  G. Hansson.  Cytokines regulate proliferation and cytoskeletal organization of vascular smooth muscle cells. , 1994, Pathology, research and practice.

[5]  C. Esmon.  Possible involvement of cytokines in diffuse intravascular coagulation and thrombosis. , 1994, Bailliere's clinical haematology.

[6]  P. Gaulard,et al.  Elevated levels of interleukin-1 beta (IL-1 beta) and IL-6 in serum and increased production of IL-1 beta mRNA in lymph nodes of patients with polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes (POEMS) syndrome. , 1994, Blood.

[7]  L. Bélec,et al.  Early weight loss and high serum tumor necrosis factor‐α levels in polyneuropathy, organomegaly, endocrinopathy, M protein, skin changes syndrome , 1994, Annals of neurology.

[8]  E. Tartour,et al.  Poems syndrome with high interleukin (IL)6 and IL1 beta serum levels, in a patient with thyroid carcinoma and melanoma. , 1994, European Journal of Cancer.

[9]  E. Kaneko,et al.  The Crow-Fukase (POEMS) syndrome with vasospastic angina. , 1994, European Neurology.

[10]  R. Ross.  The pathogenesis of atherosclerosis: a perspective for the 1990s , 1993, Nature.

[11]  W. Kuis,et al.  Castleman disease in POEMS syndrome with elevated interleukin‐6 , 1993, Cancer.

[12]  M. Kroll,et al.  Nonatheromatous arterial thrombosis. , 1993, Annual review of medicine.

[13]  J. O'fallon,et al.  Plasma-cell dyscrasia with polyneuropathy. The spectrum of POEMS syndrome. , 1992, The New England journal of medicine.

[14]  J. Matheson,et al.  POEMS syndrome with myocardial infarction: observations concerning pathogenesis and review of the literature. , 1992, Seminars in arthritis and rheumatism.

[15]  P. Libby,et al.  Cytokines and growth factors in atherogenesis. , 1992, Archives of pathology & laboratory medicine.

[16]  S. Renaud,et al.  HIV infection and immune system in genesis of coronary lesions , 1992, The Lancet.

[17]  P. Bruneval,et al.  Atheroembolism in HIV-positive individuals , 1992, The Lancet.

[18]  I. Mercier,et al.  Coronary lesions in young HIV-positive subjects at necropsy , 1992, The Lancet.

[19]  H. Shigematsu,et al.  An Autopsy Case of Crow‐Fukase (POEMS) Syndrome with a High Level of IL‐6 in the Ascites Special Reference to Glomerular Lesions , 1992, Acta pathologica japonica.

[20]  E. Kaneko,et al.  Vasospastic angina in Crow-Fukase syndrome. , 1992, American heart journal.

[21]  R. Mandler,et al.  Castleman's disease in poems syndrome with elevated interleukin‐6 , 1992, Cancer.

[22]  Y. Yuzawa,et al.  A case of POEMS syndrome showing elevated serum interleukin 6 and abnormal expression of interleukin 6 in the kidney. , 1992, Nephron.

[23]  C. Otto,et al.  Aortic valvular fibroma as a source of systemic emboli in POEMS syndrome. , 1991, Journal of the American Society of Echocardiography.

[24]  P. Libby,et al.  Involvement of the immune system in human atherogenesis: current knowledge and unanswered questions. , 1991, Laboratory investigation; a journal of technical methods and pathology.

[25]  E. Kovacs.  Fibrogenic cytokines: the role of immune mediators in the development of scar tissue. , 1991, Immunology today.

[26]  R. Aspinall,et al.  T-cell development in the fetus and the invariant series hypothesis. , 1991, Immunology today.

[27]  Cotran Rs,et al.  The Pathogenesis of Atherosclerosis: Atherogenesis and Inflammation , 1989 .

[28]  M. Bousser,et al.  Fate of the patient with chronic leg ischaemia. A review article. , 1989, The Journal of cardiovascular surgery.

[29]  J. Pober.  Warner-Lambert/Parke-Davis award lecture. Cytokine-mediated activation of vascular endothelium. Physiology and pathology. , 1988, The American journal of pathology.

[30]  C. Boitard,et al.  POEMS syndrome presenting as systemic sclerosis. Clinical and pathologic study of a case with microangiopathic glomerular lesions. , 1988, The American journal of medicine.

[31]  N. Harris,et al.  Case 10-1987: A 59-Year-Old Woman with Progressive Polyneuropathy and Monoclonal Gammopathy , 1987 .

[32]  V. Challa,et al.  Light and electron microscopic findings in POEMS, or Japanese multisystem syndrome. , 1986, Arthritis and rheumatism.

[33]  Y. Toyokura,et al.  The Crow‐Fukase syndrome , 1984, Neurology.

[34]  M. Tobin,et al.  The Japanese plasma cell dyscrasia syndrome: case report and theory of pathogenesis , 1982, Postgraduate medical journal.

[35]  Richard E. B. Solomons,et al.  Plasma Cell Dyscrasia with Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy and Skin Changes: The POEMS Syndrome , 1982, Journal of the Royal Society of Medicine.

[36]  D. Resnick,et al.  Plasma-cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes: the POEMS syndrome. Distinctive radiographic abnormalities. , 1980, Radiology.

[37]  R. Virmani,et al.  Thrombocytosis, coronary thrombosis and acute myocardial infarction. , 1979, The American journal of medicine.

[38]  A. Masi,et al.  Polyneuropathy and anasarca: evidence for a new connective-tissue syndrome and vasculopathic contribution. , 1976, Annals of internal medicine.

[39]  J. Droz,et al.  [Plasma cell dyscrasia with arteriopathy, polyneuropathy, and endocrine syndrome. A Japanese disease in an Italian patient]. , 1975, Annales de medecine interne.

[40]  D. Rushton.  Peripheral Sensorimotor Neuropathy Associated with a Localized Myeloma , 1965, British medical journal.