Light chain amyloidosis
Intraventricular dyssynchrony in light chain amyloidosis: a new mechanism of systolic dysfunction assessed by 3-dimensional echocardiography
... Light chain amyloidosis is an often fatal disease with about 2500 new cases per year in the United States [3,32- 35]. Patients with cardiac involvement have the worst out- comes in the presence of ...
7
The genomic landscape of plasma cells in systemic light chain amyloidosis.
... Systemic light chain amyloidosis (AL) is an unstable misfolded secondary or tertiary structure of a monoclonal immunoglobulin (IG) light chain, that precipitates in the extracellular ...
10
Systemic Light Chain Amyloidosis Mimicking Rheumatic Disorders
... lambda light chain systemic amyloidosis by abdominal fat pad ...for amyloidosis [15], our patient was staged as Stage II, which has median survival of 97 months in stem cell transplant ...
5
Successful management of refractory pleural effusion due to systemic immunoglobulin light chain amyloidosis by vincristine adriamycin dexamethasone chemotherapy: a case report
... immunoglobulin light chain amyloidosis presenting with bilateral pleural effusion (more severe on the right side) in the absence of cardiac decompensation that was refractory to diuretic ...
5
Genome-wide association study of immunoglobulin light chain amyloidosis in three patient cohorts: comparison with myeloma.
... Immunoglobulin light chain (AL) amyloidosis is characterized by tissue deposition of amyloid fibers derived from immunoglobulin light ...AL amyloidosis and multiple myeloma (MM) ...
32
<p>Identification of Candidate Genes and Therapeutic Agents for Light Chain Amyloidosis Based on Bioinformatics Approach</p>
... Ribosome biogenesis is a multistage process that involves transcriptional and posttranscriptional regulation, and it is over activated in cancer cells. 23,24 In our study, overexpres- sion IMP3 and FBL gene was accordant ...
10
Light-chain amyloidosis mimicking giant cell arteritis in a bilateral anterior ischemic optic neuropathy case
... was light perception in the right eye and 20/20 in the left eye. Pupil light reflex examination showed a marked relative afferent defect (RAPD) of the right ...
5
Regional myocardial microvascular dysfunction in cardiac amyloid light chain amyloidosis: assessment with 3T cardiovascular magnetic resonance
... AL amyloidosis were enrolled in the study, according to the following inclu- sion criteria: (a) AL amyloidosis, initially diagnosed in extracardiac tissue using congo red and immunohisto- chemical staining ...
11
Psychometric validation of the SF-36 Health Survey in light chain amyloidosis: results from community-based and clinic-based samples
... Background: Light chain (AL) amyloidosis, a rare and life-threatening protein misfold- ing disorder, causes organ damage and severely impacts health-related quality of life ...AL amyloidosis ...
11
Recurrent mutations of MAPK pathway genes in multiple myeloma but not in amyloid light-chain amyloidosis
... organ amyloidosis and osteolytic lesions: myeloma (n = 17), myeloma with AL amyloidosis (n = 6), and AL amyloidosis (n = ...AL amyloidosis (Table ...AL amyloidosis (n = 2), or myeloma ...
10
The burden of amyloid light chain amyloidosis on health-related quality of life
... AL amyloidosis, including heart (cardiac), kidney, liver, nervous system, and gastrointestinal ...serum-free light chains, is often used as a meas- ure of treatment efficacy for AL amyloidosis ...
10
Aberrant immunoglobulin synthesis in light chain amyloidosis Free light chain and light chain fragment production by human bone marrow cells in short term tissue culture
... with light chain amyloid (AL) deposition were examined by biosynthetic labeling ...monoclonal light chain (L-chain) synthesis even in those patients whose serum or urine contained no M ...
10
Breast amyloidosis: a case report
... Amyloidosis is an uncommon disorder characterized by extracellular deposition of abnormal proteins. Breast involvement has rarely been reported and can clinically be misdiagnosed as breast cancer. A 60-year-old ...
5
Case Report Renal amyloidosis complicated by light chain deposition nephropathy: a case report
... Abstract: Light chain amyloidosis (AL) and light chain deposition disease (LCDD) are systemic diseases caused by an abnormal production of monoclonal immunoglobulin light chains ...
5
AL amyloidosis with non-amyloid forming monoclonal immunoglobulin deposition; a case mimicking AHL amyloidosis
... “AL amyloidosis with non-amyl- oid forming monoclonal immunoglobulin deposition ” using immunostaining and two distinct LC-MS/MS ana- ...AH amyloidosis [2 – 4, 21], it was questioned whether a single ...
6
Novel renal replacement strategies for the elimination of serum free light chains in patients with kappa light chain nephropathy
... Hemodialysis with newly designed protein leaking membranes may represent an alternative strategy to clear sFLC more effectively. Due to an increased pore size of these membranes the elimination of middle sized molecules ...
8
Clinical management of gastrointestinal amyloidosis
... Colchicine, an alkaloid extracted from Colchicum autumnale of the lily family, is prescribed for treatment of diverse illnesses such as gout, Behçet’s disease and primary biliary cirrhosis [74]. This drug exerts ...
8
Gelsolin related amyloidosis Identification of the amyloid protein in Finnish hereditary amyloidosis as a fragment of variant gelsolin
... The Finnish type of familial amyloidosis is a systemic disease characterized by progressive cranial neuropathy, corneal lattice dystrophy, and distal sensimotor neuropathy. Amyloid fibrils were isolated from the ...
6
Diagnosis of secondary amyloidosis in alkaptonuria
... LSG biopsy appears as a simple and safe method to de- tect generalized amyloidosis in patients with a chronic inflammatory disease such as AKU. The results of our pilot study demonstrate that the prevalence of ...
9
Light chain (AL) amyloidosis: update on diagnosis and management
... temic amyloidosis, subcutaneous CPHPC resulted in sig- nificant decreases in the circulating SAP concentration; however, tissue-bound SAP remained present in amyloid deposits in tissues ...
8