BACKGROUND Few data are available on the clinical features and outcomes of Chinese patients with systemic immunoglobulin light-chain (AL) amyloidosis. The aim of this study is to reveal the clinical picture and risk factors of disease progression in a large cohort of Chinese patients with AL amyloidosis. METHODS Patients in the Jinling Hospital amyloidosis registry from 2003 to 2011 were stud...

Long-term survival of patients with chronic renal failure treated with regular haemodialysis has recently been recognized to be associated with peculiar complications which involve the osteoarticular system [1]. Such complications are related to a new form of systemic amyloidosis which is due to /?2-microglobulin (/J2-M), a protein which is catabolized by normal kidneys and accumulates when ren...

Amyloidosis is characterized by an abnormal extracellular deposition of amyloid in different organs, where it usually causes some type of dysfunction. Its cause is unknown. Five different types of amyloidosis have been described according to the underlying disease; immunoglobulin amyloidosis, familial amyloidosis, senile systemic amyloidosis, secondary amyloidosis and hemodialysis-associa...

Introduction AL amyloidosis is actually known to be the most common form of systemic amyloidosis in the Western countries [1, 2]. In this particular type of amyloidosis monoclonal immunoglobulin light chains, produced by plasma cell clone, undergo aggregation and form amyloid deposits, almost always systemically, and kidneys are one of the most frequent sites of amyloid deposition [3, 4, 5]. AL...

Immunoglobulin light-chain (AL) amyloidosis is caused by a usually small plasma cell clone synthesizing light chains undergoing conformational changes that lead to their aggregationanddeposition in tissues. This process leads toprogressivemultiple organdysfunction anddeath if left untreated.Advanced, irreversibleorgandamageatpresentation is the main limitation to improve theoutcomeofpatientswit...

Advanced glycation end products (AGE) are formed in long-lived matrix proteins by a nonenzymatic reaction with sugar. The presence of AGE in beta 2-microglobulin-amyloid fibrils of dialysis-related amyloidosis, one of the characteristic features of which is an accelerated bone resorption around amyloid deposits, was recently demonstrated. This suggested a potential link of AGE in bone resorptio...