title: [Light Chain Amyloidosis: an Update for Treatment].
abstract: Systemic light chain amyloidosis (AL amyloidosis) is the most common type of amyloidosis, in which deposition of misfolded monoclonal light chain secreted by underlying clonal plasma cells leads to organ dysfunction. Tissue biopsy of involved organ is needed to confirm the type of amyloid deposits, thus proper treatment could be applied. Laser microdissection followed by mass spectrometry, performed on formalin-fixed paraffin-embedded specimens, has been proven superior to traditional methods on accurate diagnosis of amyloidosis. Prognosis depends on the extent of cardiac involvement. The Mayo staging system using NT-ProBNP, cardiac troponin-T and free light chain, is the most robust method for risk stratification and treatment guidance. The introduction of autologous stem cell transplantation (auto-ASCT) resulted in long-term survival in responders, while treatment-related toxicity substantially limited the number of eligible candidates. Novel agents, especially bortezomib, thalidomide and lenalidomide hold promise to achieve comparable hematological responses with auto-ASCT, which might play significant role in treatment of recurrent or refractory AL amyloidosis.
authors.affiliation: Department of Hematology, Union Hospital of Peking Union Medical College, Beijing 100730, China.
authors.forename: Kai-Ni
authors.initials: KN
authors.lastname: Shen
authors.affiliation: Department of Hematology, Union Hospital of Peking Union Medical College, Beijing 100730, China. E-mail: pumch.li.jian@gmail.com.
authors.forename: Jian
authors.initials: J
authors.lastname: Li
mesh_terms: D000686:Amyloidosis; D000069286:Bortezomib; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007147:Immunoglobulin Light Chains; D000075363:Immunoglobulin Light-chain Amyloidosis; D020097:Natriuretic Peptide, Brain; D010446:Peptide Fragments; D011379:Prognosis
chemical_list: D007147:Immunoglobulin Light Chains; D010446:Peptide Fragments; C109794:pro-brain natriuretic peptide (1-76); D020097:Natriuretic Peptide, Brain; D000069286:Bortezomib