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...

primary localized amyloidosis of eyelid is a localized type of amyloidosis without evidence of systemic involvement. we report a 75-year-old man suffering of unilateral (left) ptosis due to upper eyelid mass with bony consistency. eye examination revealed upper lid mass attached to tarsus. no ocular infection or inflammation was found. pathologist reported diffuse distribution of eosinophilic h...

Gastrointestinal amyloidosis (GIA), a protein deposition disorder, represents a complex common pathway that encompasses multiple etiologies and presentations. It represents a significant diagnostic and treatment challenge. The disease results from the deposition of insoluble extracellular protein fragments that have been rendered resistant to digestion. GIA can be acquired or genetic, and most ...

We read with interest the pictorial essay by Özcan et al. (1) entitled “Imaging for abdominal involvement in amyloidosis” in the recent issue of Diagnostic and Interventional Radiology. In this article, the authors provide both textual and visual portrayals of abdominal amyloidosis, which is difficult to diagnose because of nonspecific imaging findings and frequent subclinical presentation. Bot...

Background: Light chain (AL) amyloidosis is a plasma cell disorder characterized by abnormal fibrillary light deposition causing cardiac, renal, hepatic, gastrointestinal and peripheral nervous system dysfunction. Muscle disease occurs in 1.5% of individuals progressive proximal weakness thus far considered untreatable. Methods: We reviewed two cases AL associated myopathy at our institution wh...

purpose : primary localized amyloidosis of eyelid is a localized type of amyloidosis without evidence of systemic involvement, which is a quite rare clinical condition. here we report a case of primary localized amyloidosis of eyelid. case report : a 40-year-old woman presented with a four-month history of swelling of the left upper eyelid resulting in a mechanical ptosis. the mass had a firm b...

The first case of amyloidosis is reported in which spontaneous massive hepatic haemorrhage necessitated emergency liver transplantation. Liver transplantation, as a treatment for a failing liver due to amyloidosis has not been previously reported. At transplantation, the liver tissue was uncharacteristically friable, although the subsequent vascular and biliary anastomoses were uncomplicated. H...