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 …

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 …

Recent improvements in survival in primary systemic amyloidosis and the importance of an early mortality risk score

SK Kumar, MA Gertz, MQ Lacy, D Dingli… - Mayo Clinic …, 2011 - Elsevier
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 …

[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 …

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 …

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 …

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 …

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 …

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 …

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 …