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Definition of organ involvement and treatment response in immunoglobulin light chain amyloidosis (AL): A consensus opinion from the 10th International Symposium on Amyloid and Amyloidosis

TLDR
A consensus panel was convened comprising 13 specialists actively involved in the treatment of patients with amyloidosis, and a consensus was developed defining each organ involved and the criteria for response.
Abstract
We undertook this study to develop uniformly accepted criteria for the definition of organ involvement and response for patients on treatment protocols for immunoglobulin light-chain amyloidosis (AL). A consensus panel was convened comprising 13 specialists actively involved in the treatment of patients with amyloidosis. Institutional criteria were submitted from each, and a consensus was developed defining each organ involved and the criteria for response. Specific criteria have been developed with agreed on definitions of organ and hematologic response as a result of discussions at the 10th International Symposium on Amyloid and Amyloidosis held in Tours, France, April 2004. These criteria now form the working definition of involvement and response for the purposes of future data collection and reporting. We report criteria that centers can now use to define organ involvement and uniform response criteria for reporting outcomes in patients with light-chain AL.

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Natural history and outcome in systemic AA amyloidosis.

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Classification of amyloidosis by laser microdissection and mass spectrometry-based proteomic analysis in clinical biopsy specimens.

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References
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Journal ArticleDOI

Criteria for evaluating disease response and progression in patients with multiple myeloma treated by high-dose therapy and haemopoietic stem cell transplantation. Myeloma Subcommittee of the EBMT. European Group for Blood and Marrow Transplant.

TL;DR: New criteria for response and progression have been developed as a result of discussions between representatives of the Myeloma Subcommittee of the Chronic Leukaemia Working Party of the European Group for Blood and Marrow Transplantation (EBMT) and representatives ofThe Myelomas Working Committee of the Autologousblood and marrow transplant Registry (ABMTR) and the International Bone Marrowtransplant Registry (IBMTR).
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A long-term study of prognosis in monoclonal gammopathy of undetermined significance.

TL;DR: The risk of progression of MGUS to multiple myeloma or related disorders is about 1 percent per year, and the initial concentration of serum monoclonal protein was a significant predictor of progression at 20 years.
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Review: History of the Amyloid Fibril

TL;DR: Chemical and physical definition of the fibrils in tissues will be needed to relate the in vitro properties of amyloid protein fibril formation in vivo to the pathogenesis ofAmyloid fibrillar formation in vitro.
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Amyloid cardiomyopathy: Characterization by a distinctive voltage/mass relation

TL;DR: There was an inverse correlation between voltage and muscle cross-sectional area in patients with amyloidosis; moreover, marked derangement of the voltage/cross-sectional Area relation was associated with clinical symptoms and mortality.
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Serum Reference Intervals and Diagnostic Ranges for Free κ and Free λ Immunoglobulin Light Chains: Relative Sensitivity for Detection of Monoclonal Light Chains

TL;DR: Reference and diagnostic intervals for serum FLCs have been developed for use with a new, automated immunoassay that makes the detection and quantification of monoclonal F LCs easier and more sensitive than with current methods.
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