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The challenge of systemic immunoglobulin light-chain amyloidosis (Al)

PALLADINI, GIOVANNI;
2012-01-01
2012
Protein Aggregation and Fibrillogenesis in Cerebral and Systemic Amyloid Disease
Medical Research, General Topics covers a wide array of topics in medical and biomedical research, with a specific emphasis on human disease, human tissues, and all levels of research into the pathogenesis of clinically significant conditions. Specific medical fields that are characterized by the inclusion of material from several other specializations are also covered here; these include general and internal medicine, tropical medicine, pediatrics, gerontology, epidemiology, and public health. Resources dealing with specific clinical interventions are excluded and are placed in the Medical Research: Diagnosis & Treatment category. Resources that emphasize the specific disease types, or specific systems affected are also excluded and are categorized according to the pathogen or system pathophysiology.
SUBCELLULAR BIOCHEMISTRY
2-s2.0-84889658374
Esperti anonimi
Inglese
Internazionale
STAMPA
65
609
642
34
The cardiac involvement and associated mortality that occur in systemic AL amyloidosis remain among the most challenging aspects of the systemic amyloid-related diseases. Monoclonal immunoglobulin light chains produced by a clone of plasma cells are usually the cause of symptoms and organ dysfunction via both poorly understood toxic effects of misfolded species and accumulation of interstitial amyloid fibrils in key viscera. Treatment is aimed at eliminating the clonal cells in order to eliminate toxic light chain production. Recent advances in therapy have helped many patients with AL achieve complete hematologic responses and significant reversal of organ damage but these benefits do not extend to that 10-15 % who present with advanced cardiac involvement. Even with cardiac transplant followed by effective therapy such as stem cell transplant, outcomes for these patients remain promising at best.
Medicine (all)
http://www.ncbi.nlm.nih.gov/pubmed/23225018
sì
2 Contributo in Volume::2.1 Contributo in volume (Capitolo o Saggio)
2
268
none
Palladini, Giovanni; Comenzo, Raymond L.
info:eu-repo/semantics/bookPart
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11571/1106175
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