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[Immunoglobulin light-chain type amyloidosis].

Nihon Naika Gakkai zasshi. The Journal of the Japanese Society of Internal Medicine, 1995
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Immunoglobulin light chain amyloidosis

Expert Review of Hematology, 2013
Primary light chain amyloidosis is the most common form of systemic amyloidosis and is caused by misfolded light chains that cause proteotoxicity and rapid decline of vital organ function. Early diagnosis is essential in order to deliver effective therapy and prevent irreversible organ damage.
MERLINI, GIAMPAOLO, Comenzo RL, Seldin DC, Wechalekar A, Gertz MA   +4 more
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Immunoglobulin Light Chain Systemic Amyloidosis

Cancer Treatment and Research, 2016
Immunoglobulin light chain amyloidosis (AL) is a rare, complex disease caused by misfolded free light chains produced by a usually small, indolent plasma cell clone. Effective treatments exist that can alter the natural history, provided that they are started before irreversible organ damage has occurred.
Giampaolo Merlini, Angela Dispenzieri
exaly   +3 more sources

Glycosylation of immunoglobulin light chains associated with amyloidosis

Amyloid: the International Journal of Experimental and Clinical Investigation: the Official Journal of the International Society of Amyloidosis, 2000
AL amyloidosis is a fatal disease caused by deposition of immunoglobulin light chains in a fibrillarforin (AL) in various organs. By searching the Kabat database of immunoglobulin sequences using the KabatMan software, we have shown that there is a preponderance of the consensus glycosylation sequon (AsnXxxSer/Thr) in the framework regions of amyloid ...
Svein Haavik, Elizabeth F Hounsell, D V Renouf   +2 more
exaly   +3 more sources

Immunohistochemical study of immunoglobulin light chain amyloidosis with antibodies to the immunoglobulin light chain variable region

Pathology International, 2006
To detect immunoglobulin (Ig) light chain amyloidosis (AL amyloidosis) in formalin‐fixed, paraffin‐embedded tissue sections by immunohistochemistry, polyclonal antibodies were generated against synthetic peptides corresponding to amino acids 1–19 of the Ig λ light chain V λ VI subgroup (anti‐V λ VI (1–19)) and the Ig κ light chain Vκ I subgroup (anti ...
Yoshinobu, Hoshii, Makiko, Kiyama, Dan, Cui, Hiroo, Kawano, Tokuhiro, Ishihara   +4 more
exaly   +3 more sources

Therapy for immunoglobulin light chain amyloidosis: the new and the old

Blood Reviews, 2004
An accurate diagnosis of amyloidosis and its subtype classification are essential for disease prognostication and treatment. In primary amyloidosis, overall median survival is approximately 2 years and may be less in patients with cardiomyopathy. Current therapy for primary amyloidosis is suboptimal.
Angela Dispenzieri, Morie Gertz, Martha Lacy   +2 more
exaly   +3 more sources

The Challenge of Systemic Immunoglobulin Light-Chain Amyloidosis (AL)

, 2012
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 ...
PALLADINI, GIOVANNI, Comenzo, Raymond L.
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Proposed Cardiac End Points for Clinical Trials in Immunoglobulin Light Chain Amyloidosis: Report From the Amyloidosis Forum Cardiac Working Group [PDF]

Circulation: Heart Failure, 2022
Immunoglobulin light chain amyloidosis is a rare, multisystemic, phenotypically heterogenous disease affecting cardiovascular, renal, neurological, and gastrointestinal systems to varying degrees.
Mathew Maurer, Giampaolo Merlini, Amyloidsis Forum   +2 more
exaly   +3 more sources

Nonchemotherapy Treatment of Immunoglobulin Light Chain Amyloidosis

Acta Haematologica, 2020
Immunoglobulin light chain amyloidosis (AL amyloidosis) is a rare, life-threatening disease characterized by the deposition of misfolded proteins in vital organs such as the heart, the lungs, the kidneys, the peripheral nervous system, and the gastrointestinal tract. This causes a direct toxic effect, eventually leading to organ failure. The underlying
Layla, Van Doren, Suzanne, Lentzsch
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