title: Light chain amyloidosis 2012: a new era.
abstract: AL amyloidosis patients with multi-organ and particularly cardiac involvement have historically been considered to have a bad prognosis. The introduction of autologous stem cell transplantation was associated with unacceptable toxicity in high-risk patients, but responding patients have prolonged overall survival. Toxicities can be decreased by careful patient selection, but this reduces the applicability of this treatment modality to a limited number of patients. Efforts are therefore needed to design novel more effective regimens, with the use of new medications, such as thalidomide, lenalidomide and bortezomib, next generation immunomodulatory drugs and proteasome inhibitors. Their combination with dexamethasone and alkylating agents show promising results, allowing a high percentage of remission and subsequent event-free and overall survival, even in a significant proportion of high risk, poor prognosis populations. This review includes the state-of-the-art treatment for AL amyloidosis patients as of 2012, in light of the progress in management of this disease during recent years.
authors.affiliation: Department of Haematology, Hadassah Hebrew University Medical Centre, Jerusalem, Israel. rmoshg@hadassah.org.il
authors.forename: Moshe E
authors.initials: ME
authors.lastname: Gatt
authors.forename: Giovanni
authors.initials: G
authors.lastname: Palladini
mesh_terms: D000477:Alkylating Agents / Q000627:therapeutic use*; D000682:Amyloid / Q000378:metabolism*; D000686:Amyloidosis / Q000150:complications / Q000188:drug therapy* / Q000378:metabolism / Q000473:pathology / Q000601:surgery; D015415:Biomarkers; D001897:Boronic Acids / Q000627:therapeutic use; D000069286:Bortezomib; D009202:Cardiomyopathies / Q000097:blood / Q000209:etiology / Q000473:pathology; D002986:Clinical Trials as Topic; D003131:Combined Modality Therapy; D003907:Dexamethasone / Q000627:therapeutic use; D005544:Forecasting; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000378:metabolism*; D007155:Immunologic Factors / Q000627:therapeutic use*; D020097:Natriuretic Peptide, Brain / Q000097:blood; D010446:Peptide Fragments / Q000097:blood; D011379:Prognosis; D011719:Pyrazines / Q000627:therapeutic use; D012306:Risk; D012720:Severity of Illness Index; D033581:Stem Cell Transplantation; D019209:Troponin C / Q000097:blood; D020107:Troponin T / Q000097:blood
chemical_list: D000477:Alkylating Agents; D000682:Amyloid; D015415:Biomarkers; D001897:Boronic Acids; D007147:Immunoglobulin Light Chains; D007155:Immunologic Factors; D010446:Peptide Fragments; D011719:Pyrazines; D019209:Troponin C; D020107:Troponin T; C035222:amyloid protein AL; C109794:pro-brain natriuretic peptide (1-76); D020097:Natriuretic Peptide, Brain; D000069286:Bortezomib; D003907:Dexamethasone