Light chain AL amyloidosis: update on diagnosis and managementReport as inadecuate




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Journal of Hematology and Oncology

, 4:47

First Online: 18 November 2011Received: 18 October 2011Accepted: 18 November 2011

Abstract

Light chain AL amyloidosis is a plasma cell dyscrasia characterized by the pathologic production of fibrillar proteins comprised of monoclonal light chains which deposit in tissues and cause organ dysfunction. The diagnosis can be challenging, requiring a biopsy and often specialized testing to confirm the subtype of systemic disease. The goal of treatment is eradication of the monoclonal plasma cell population and suppression of the pathologic light chains which can result in organ improvement and extend patient survival. Standard treatment approaches include high dose melphalan HDM followed by autologous hematopoietic stem cell transplantation SCT or oral melphalan with dexamethasone MDex. The use of novel agents thalidomide, lenalidomide and bortezomib alone and in combination with steroids and alkylating agents has shown efficacy and continues to be explored. A risk adapted approach to SCT followed by novel agents as consolidation reduces treatment related mortality with promising outcomes. Immunotherapeutic approaches targeting pathologic plasma cells and amyloid precursor proteins or fibrils are being developed. Referral of patients to specialized centers focusing on AL amyloidosis and conducting clinical trials is essential to improving patient outcomes.

Electronic supplementary materialThe online version of this article doi:10.1186-1756-8722-4-47 contains supplementary material, which is available to authorized users.

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Author: Michael Rosenzweig - Heather Landau

Source: https://link.springer.com/







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