Le syndrome POEMS (ou syndrome de Crow-Fukase)

Lagueny, A; Bouillot, Sandrine; Vital, C; Ferrer, Xavier; Larrieu, Jean-Marc; Vital, Anne

doi:10.1016/s0035-3787(04)70903-0

Cited by 11 publications

(3 citation statements)

References 89 publications

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“…Les nerfs crâniens sont épargnés et il n'existe pas de dysautonomie. Les examens complémentaires sont en faveur d'une neuropathie démyélinisante [2][3][4][5]. La ponction lombaire trouve une hyperprotéinorachie sans réaction cellulaire dans presque tous les cas.…”

Section: Neuropathieunclassified

“…La biopsie neuromusculaire montre des lésions démyélinisantes segmentaires et des lésions axonales, fréquemment accompagnées d'une importante raréfaction des fibres myélinisées. Les lamelles de myéline non compactes qui doivent être différenciées de l'élar-gissement des lamelles de myéline observé dans les neuropathies à IgM à activité antiMAG (Myelin Associated Glycoprotein) sont fréquentes au cours du syndrome POEMS mais ne sont cependant pas spécifiques [5].…”

Section: Neuropathieunclassified

“…L'hyperpigmentation est la plus fréquente des manifestations cutanées [2][3][4][5] généralisée mais qui peut être localisée. L'hypertrichose prédomine sur la face, le thorax et les membres [3].…”

Section: Manifestations Cutanéesunclassified

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Syndrome POEMS

Soubrier

2007

La Presse Médicale

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The POEMS syndrome combines a constant polyneuropathy (P), organomegaly (O), endocrinopathy (E), monoclonal gammopathy (M) (or other plasma cell disorder) and skin changes (S). Other manifestations may be observed: anasarca, fever, sweating, clubbed fingers, renal damage, arterial obstruction, pulmonary hypertension, thrombocytosis, and polycythemia. Its pathogenesis is not well elucidated but elevated levels of vascular endothelial growth factor (VEGF) appear to characterize it. Consistent plasma cell disorders include a monoclonal component, often in small quantities with a lambda light chain isotype, and plasmacytoma, often solitary lesions. Treatment depends on specific characteristics of the disease and the patient (radiation therapy for plasmocytoma, autologous bone marrow transplantation in young subjects, corticosteroid therapy or chemotherapy in the elderly). The usefulness of thalidomide and bevacuzimab in refractory POEMS syndromes remains to be seen.

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Section: Neuropathieunclassified

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Syndrome POEMS

Soubrier

2007

La Presse Médicale

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POEMS syndrome in a 15-year-old boy: radiological findings

2007

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POEMS syndrome is a multisystem disorder related to a plasma-cell dyscrasia. It is usually seen between the ages of 40 and 50 years and is very rare in childhood. Radiologically, this syndrome is characterized by sclerotic focal bone lesions with a normal radionuclide bone scan. We report a case of POEMS syndrome in a 15-year-old boy in whom plain radiographs and multidetector-row spiral CT (MDCT) provided information that was helpful in the clinical assessment.

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Peripheral Neuropathies

Lunn

Sheikh²

2014

The Autoimmune Diseases

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Le syndrome POEMS (ou syndrome de Crow-Fukase)

Cited by 11 publications

References 89 publications

Syndrome POEMS

Syndrome POEMS

POEMS syndrome in a 15-year-old boy: radiological findings

Peripheral Neuropathies

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