Systemic amyloidosis recognition, prognosis, and therapy: a systematic review
MA Gertz, A Dispenzieri - Jama, 2020 - jamanetwork.com
Importance Many patients with systemic amyloidosis are underdiagnosed. Overall, 25% of
patients with immunoglobulin light chain (AL) amyloidosis die within 6 months of diagnosis …
patients with immunoglobulin light chain (AL) amyloidosis die within 6 months of diagnosis …
New developments in diagnosis, risk assessment and management in systemic amyloidosis
I Vaxman, A Dispenzieri, E Muchtar, M Gertz - Blood reviews, 2020 - Elsevier
Amyloidosis is a group of disorders characterized by a misfolded protein that deposits in
organs and compromise their function. Clinician should have a high index of suspicion …
organs and compromise their function. Clinician should have a high index of suspicion …
Recent improvements in survival in primary systemic amyloidosis and the importance of an early mortality risk score
OBJECTIVE To examine whether the outcome of patients with primary systemic amyloidosis
(AL) has improved over time and to identify predictors of early mortality in patients with AL …
(AL) has improved over time and to identify predictors of early mortality in patients with AL …
[HTML][HTML] Amyloidosis—the diagnosis and treatment of an underdiagnosed disease
S Ihne, C Morbach, C Sommer, A Geier… - Deutsches Ärzteblatt …, 2020 - ncbi.nlm.nih.gov
Background Systemic amyloidosis is a multi-system disease caused by fibrillary protein
deposition with ensuing dysfunction of the affected organ systems. Its diagnosis is often …
deposition with ensuing dysfunction of the affected organ systems. Its diagnosis is often …
The prevalence and management of systemic amyloidosis in western countries
HLA Nienhuis, J Bijzet, BPC Hazenberg - Kidney Diseases, 2016 - karger.com
Background: Amyloidosis has been a mystery for centuries, but research of the last decennia
has clarified many of the secrets of this group of diseases. A protein-based classification of …
has clarified many of the secrets of this group of diseases. A protein-based classification of …
Systemic amyloidosis: novel therapies and role of biomarkers
M Nuvolone, G Merlini - Nephrology Dialysis Transplantation, 2017 - academic.oup.com
Systemic amyloidosis is caused by misfolding and extracellular deposition of one of an ever-
growing list of circulating proteins, resulting in vital organ dysfunction and eventually death …
growing list of circulating proteins, resulting in vital organ dysfunction and eventually death …
Changing epidemiology of AA amyloidosis: clinical observations over 25 years at a single national referral centre
T Lane, JH Pinney, JA Gilbertson, DF Hutt… - Amyloid, 2017 - Taylor & Francis
Objective: Systemic AA amyloidosis is a serious complication of chronic inflammation;
however, there are relatively few published data on its incidence. We investigated the …
however, there are relatively few published data on its incidence. We investigated the …
Pathophysiology and treatment of systemic amyloidosis
JD Gillmore, PN Hawkins - Nature Reviews Nephrology, 2013 - nature.com
Amyloid is an abnormal extracellular fibrillar protein deposit in the tissues. In humans, more
than 25 different proteins can adopt a fibrillar conformation in vivo that results in the …
than 25 different proteins can adopt a fibrillar conformation in vivo that results in the …
Marked progress in AL amyloidosis survival: a 40-year longitudinal natural history study
A Staron, L Zheng, G Doros, LH Connors… - Blood cancer …, 2021 - nature.com
The recent decades have ushered in considerable advancements in the diagnosis and
treatment of systemic light chain (AL) amyloidosis. As disease outcomes improve, AL …
treatment of systemic light chain (AL) amyloidosis. As disease outcomes improve, AL …
A European collaborative study of cyclophosphamide, bortezomib, and dexamethasone in upfront treatment of systemic AL amyloidosis
G Palladini, S Sachchithanantham… - Blood, The Journal …, 2015 - ashpublications.org
The combination of cyclophosphamide/bortezomib/dexamethasone (CyBorD) showed early
promise of high rates of hematologic responses tempered by studies showing the inability to …
promise of high rates of hematologic responses tempered by studies showing the inability to …