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Published on: May 19, 2020
Localized amyloid light-chain amyloidosis and extramedullary plasmacytoma of the mitral valve
Aurélien Roumy1, Laurence de Leval, Lars Niclauss
1Department of Cardiovascular Surgery, University Hospital of Lausanne, VD, Switzerland. aurelien.roumy@chuv.ch
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.
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