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[New elements in the diagnosis and the treatment of primary AL amyloid polyneuropathy and neuropathy due to POEMS syndrome]. / Nouveautés dans le diagnostic et le traitement des neuropathies périphériques liées à l'amylose AL et au syndrome POEMS.
Adams, D; Lozeron, P; Theaudin, M; Ribrag, V; Bourhis, J-H; Lacroix, C.
Afiliação
  • Adams D; Service de neurologie, CHU de Bicêtre, AP-HP, université Paris-Sud 11, 78, rue du Général-Leclerc, 94275 Le Kremlin Bicêtre, France. david.adams@bct.aphp.fr
Rev Neurol (Paris) ; 167(1): 57-63, 2011 Jan.
Article em Fr | MEDLINE | ID: mdl-21190703
INTRODUCTION: Primary AL amyloid polyneuropathy (AL-PN) and neuropathy due to POEMS syndrome (POEMS-N) are rare, associated with a monoclonal gammopathy (MG) IgGλ or IgAλ at a low rate and systemic manifestations. They are invalidating and life-threatening. STATE OF THE ART: AL-PN usually mimics small fiber length-dependent axonal polyneuropathies, but also multifocal or painful neuropathies, POEMS-N corresponds to a rapid ascending CIDP with MG. To confirm the diagnosis of AL-PN, initial investigations should identify amyloidosis on nerve or accessory salivary glands, to establish the type of amyloid after serum free light-chain (FLC) measurements. For the diagnosis of N-POEMS, diagnosis is based on the presence of four criteria proposed by Dispenzieri. These neuropathies are associated with biomarkers, useful for diagnosis and treatment monitoring: elevated serum level of FLC monoclonal in (AL-PN) or VEGF (N-POEMS). PERSPECTIVES: Early diagnosis of these neuropathies and early treatment using high-dose melphalan associated with an autologous hematopoietic stem cell graft or low monthly doses can improve the clinical manifestations and patient survival. CONCLUSIONS: Systematic search for monoclonal gammopathy by immunofixation and serum free light chains is very useful for the management of progressive peripheral neuropathies of unknown origin.
Assuntos

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Síndrome POEMS / Neuropatias Amiloides / Amiloide Tipo de estudo: Diagnostic_studies / Etiology_studies / Prognostic_studies / Screening_studies Limite: Humans Idioma: Fr Revista: Rev Neurol (Paris) Ano de publicação: 2011 Tipo de documento: Article

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Síndrome POEMS / Neuropatias Amiloides / Amiloide Tipo de estudo: Diagnostic_studies / Etiology_studies / Prognostic_studies / Screening_studies Limite: Humans Idioma: Fr Revista: Rev Neurol (Paris) Ano de publicação: 2011 Tipo de documento: Article