Le POEMS syndrome

Rose, Cameron.; Mahieu, Maurice; Hachulla, É.; Facon, Thierry; Hatron, P.Y.; Bauters, F; Devulder, B

doi:10.1016/s0248-8663(97)80807-7

Cited by 18 publications

(23 citation statements)

References 58 publications

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“…This affection seems to be more frequent in the Japanese population, demonstrates a male predominance and mostly occurs between the age of 40 and 50 [1,[4][5][6].…”

Section: Discussionmentioning

confidence: 99%

“…Lesions are habitually osteosclerotic, or both osteolytic and osteosclerotic [1,[4][5][6]. Monoclonal gammopathy is always found in POEMS syndrome, usually IgA or IgG, and less frequently light chains.…”

Section: Discussionmentioning

confidence: 99%

“…POEMS syndrome (Crow-Fukase, Takatsuki, polyneuropathy-endocrinopathy-plasma cell dyscrasia syndrome) is a multisystem disorder, associated with plasma cell dyscrasia, and characterized by polyneuropathy (P), organomegaly (O), endocrinopathy (E), monoclonal protein (M) and skin changes (S) [1]. Glomeruloid angioma is a recently described distinctive histological entity considered to be a specific marker of POEMS syndrome [2].…”

Section: Introductionmentioning

confidence: 99%

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POEMS Syndrome Revealed by Multiple Glomeruloid Angiomas

et al. 2002

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POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin disorders) is a rare multisystemic disease associated with plasma cell dyscrasia. A 68-year-old woman with chronic renal insufficiency and arterial hypertension included in her medical history was admitted to the hospital with confusion, somnolence and asthenia. She presented ascites, hepatosplenomegaly, leg oedema, distal dysesthesias, leuconychia and multiple nodular purple red angiomas on the trunk, upper limbs and fingers. Hypothyroidism was revealed in the laboratory investigations and monoclonal IgG peak in immunoelectrophoresis. Electromyography showed both demyelinisating and axonal degenerative neuropathy. The diagnosis of POEMS syndrome was based on the dermatopathological examination of a cutaneous angioma; histology revealed features of glomeruloid angioma, a specific marker of this syndrome.

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“…This affection seems to be more frequent in the Japanese population, demonstrates a male predominance and mostly occurs between the age of 40 and 50 [1,[4][5][6].…”

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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POEMS Syndrome Revealed by Multiple Glomeruloid Angiomas

et al. 2002

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“…The patient may sometimes experience nonspecific disturbances similar to B symptoms such as weight loss, loss of appetite, night sweats, fever that is sometimes accompanied by increased erythrocyte sedimentation rate, increase of serum C-reactive protein concentration and polyclonal gammopathy on serum protein electrophoresis. All these symptoms are the result of increased IL-6 production, and are almost exclusively associated with the plasmacellular histologic subtype 3,8 . Therapy of choice for unicentric form of the disease is total surgical excision of the tumor with very low incidence of relapse 8,9 .…”

mentioning

confidence: 99%

“…All these symptoms are the result of increased IL-6 production, and are almost exclusively associated with the plasmacellular histologic subtype 3,8 . Therapy of choice for unicentric form of the disease is total surgical excision of the tumor with very low incidence of relapse 8,9 . For the disease that cannot be completely excised, radiation therapy is recommended 10,11 .…”

mentioning

confidence: 99%