title: Localized amyloid light-chain amyloidosis and extramedullary plasmacytoma of the mitral valve.
abstract: An unusual case of localized amyloid light-chain (AL) amyloidosis and extramedullary plasmacytoma of the mitral valve is described. The worsening of a mitral regurgitation led to investigations and surgery. The valve presented marked distortion and thickening by type AL amyloid associated with a monotypic CD138+ immunoglobulin lambda plasma cell proliferation. Systemic staging showed a normal bone marrow and no evidence of amyloid deposition in other localizations. The patient's outcome after mitral valve replacement was excellent. To our knowledge, this is the first description of a localized AL amyloidosis as well as of a primary extramedullary plasmacytoma of the mitral valve.
authors.affiliation: Department of Cardiovascular Surgery, University Hospital of Lausanne, VD, Switzerland. aurelien.roumy@chuv.ch
authors.forename: Aurélien
authors.initials: A
authors.lastname: Roumy
authors.forename: Laurence
authors.initials: L
authors.lastname: de Leval
authors.forename: Lars
authors.initials: L
authors.lastname: Niclauss
authors.forename: Stephan C
authors.initials: SC
authors.lastname: Schaefer
authors.forename: Paul
authors.initials: P
authors.lastname: Kurtin
authors.forename: Ahmet
authors.initials: A
authors.lastname: Dogan
authors.forename: Ludwig K
authors.initials: LK
authors.lastname: von Segesser
authors.forename: Patrick
authors.initials: P
authors.lastname: Ruchat
mesh_terms: D000368:Aged; D000686:Amyloidosis / Q000473:pathology*; D005260:Female; D006338:Heart Neoplasms / Q000473:pathology*; D006349:Heart Valve Diseases / Q000473:pathology*; D006801:Humans; D008943:Mitral Valve / Q000473:pathology*; D010954:Plasmacytoma / Q000473:pathology*