title: [New elements in the diagnosis and the treatment of primary AL amyloid polyneuropathy and neuropathy due to POEMS syndrome].
abstract: BACKGROUND
  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.
  
  
  BACKGROUND
  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).
  
  
  CONCLUSIONS
  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.
authors.affiliation: 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
authors.forename: D
authors.initials: D
authors.lastname: Adams
authors.forename: P
authors.initials: P
authors.lastname: Lozeron
authors.forename: M
authors.initials: M
authors.lastname: Theaudin
authors.forename: V
authors.initials: V
authors.lastname: Ribrag
authors.forename: J-H
authors.initials: JH
authors.lastname: Bourhis
authors.forename: C
authors.initials: C
authors.lastname: Lacroix
mesh_terms: D000682:Amyloid / Q000378:metabolism*; D017772:Amyloid Neuropathies / Q000175:diagnosis* / Q000188:drug therapy* / Q000209:etiology / Q000601:surgery; D015415:Biomarkers; D001706:Biopsy; D005871:Castleman Disease / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology; D003131:Combined Modality Therapy; D004359:Drug Therapy, Combination; D042241:Early Diagnosis; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007070:Immunoglobulin A / Q000378:metabolism; D007074:Immunoglobulin G / Q000378:metabolism; D007146:Immunoglobulin lambda-Chains / Q000378:metabolism; D008558:Melphalan / Q000627:therapeutic use; D016878:POEMS Syndrome / Q000175:diagnosis* / Q000188:drug therapy / Q000378:metabolism / Q000532:radiotherapy; D010265:Paraproteinemias / Q000150:complications; D010525:Peripheral Nerves / Q000473:pathology; D011241:Prednisone / Q000627:therapeutic use; D012470:Salivary Glands, Minor / Q000473:pathology; D012867:Skin / Q000473:pathology; D013792:Thalidomide / Q000627:therapeutic use; D042461:Vascular Endothelial Growth Factor A / Q000097:blood
chemical_list: D000682:Amyloid; D015415:Biomarkers; D007070:Immunoglobulin A; D007074:Immunoglobulin G; D007146:Immunoglobulin lambda-Chains; C467484:VEGFA protein, human; D042461:Vascular Endothelial Growth Factor A; C035222:amyloid protein AL; D013792:Thalidomide; D008558:Melphalan; D011241:Prednisone