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.

Light chain amyloidosis 2012: a new era.

PALLADINI, GIOVANNI
2013-01-01

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.
2013
The Hematology category covers resources concerned with blood, blood-forming tissues, bone marrow, plasma, and transfusions. Coverage also includes resources on specialties such as hemophilia, leukemia, and lymphoma.
Esperti anonimi
Inglese
Internazionale
STAMPA
160
5
582
598
17
Alkylating Agents; therapeutic use, Amyloid; metabolism, Amyloidosis; complications/drug therapy/metabolism/pathology/surgery, Biological Markers, Boronic Acids; therapeutic use, Cardiomyopathies; blood/etiology/pathology, Clinical Trials as Topic, Combined Modality Therapy, Dexamethasone; therapeutic use, Forecasting, Humans, Immunoglobulin Light Chains; metabolism, Immunologic Factors; therapeutic use, Natriuretic Peptide; Brain; blood, Peptide Fragments; blood, Prognosis, Pyrazines; therapeutic use, Risk, Severity of Illness Index, Stem Cell Transplantation, Troponin C; blood, Troponin T; blood
http://dx.doi.org/10.1111/bjh.12191
http://onlinelibrary.wiley.com/doi/10.1111/bjh.12191/epdf
2
info:eu-repo/semantics/article
262
M. E., Gatt; Palladini, Giovanni
1 Contributo su Rivista::1.1 Articolo in rivista
none
File in questo prodotto:
Non ci sono file associati a questo prodotto.

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11571/856772
Citazioni
  • ???jsp.display-item.citation.pmc??? 16
  • Scopus 43
  • ???jsp.display-item.citation.isi??? 41
social impact