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Systemic immunoglobulin light chain amyloidosis [PDF]
Nature Reviews Disease Primers, 2018 Systemic immunoglobulin light chain amyloidosis is a protein misfolding disease caused by the conversion of immunoglobulin light chains from their soluble functional states into highly organized amyloid fibrillar aggregates that lead to organ dysfunction.
G. Merlini +6 more
semanticscholar +9 more sources
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Mayo Clinic Proceedings, 2016
OBJECTIVE To characterize the natural history of immunoglobulin light chain amyloidosis-associated myopathy and to provide guidelines for recognition.
Eli Muchtar +2 more
exaly +2 more sources
OBJECTIVE To characterize the natural history of immunoglobulin light chain amyloidosis-associated myopathy and to provide guidelines for recognition.
Eli Muchtar +2 more
exaly +2 more sources
Expert Review of Hematology, 2020 Introduction Immunoglobulin light chain (AL) amyloidosis is one of the most frequent systemic amyloidosis in Western countries. It is caused by a B-cell clone producing a misfolded light chain (LC) that deposits in organs.
M. Basset +3 more
semanticscholar +2 more sources
Immunoglobulin light chain amyloidosis: 2024 update on diagnosis, prognosis, and treatment
American journal of hematology/oncology, 2023Immunoglobulin light chain amyloidosis is a clonal, nonproliferative plasma cell disorder in which fragments of immunoglobulin light or heavy chain are deposited in tissues.
M. Gertz
semanticscholar +1 more source
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 +2 more
exaly +3 more sources
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 +2 more
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, 2000AL 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 ...
Nikolay A Paramonov +2 more
exaly +3 more sources
Treatment of immunoglobulin light chain amyloidosis
Current Hematologic Malignancy Reports, 2009No therapy is uniformly effective in the management of immunoglobulin light chain amyloidosis (AL amyloidosis). Despite the common generalization, therapy is highly effective. Options available to patients with AL amyloidosis include high-dose therapy, but this is applicable to only about one fourth of patients.
Morie A, Gertz, Steven R, Zeldenrust
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Free Light Chains of Immunoglobulins in Amyloidosis
Acta Medica Scandinavica, 1979ABSTRACT Monomeric (M) and dimeric (D) forms of free light chains in serum have been measured by a sensitive radioimmunoassay in eight patients with amyloidosis without monoclonal proteins. Significantly elevated concentrations of D lambda chains were demonstrated in two of four patients with localized amyloidosis.
J, Sølling, K, Sølling
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Immunoglobulin Light Chain Systemic Amyloidosis
2016Immunoglobulin 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.
Angela, Dispenzieri, Giampaolo, Merlini
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Systemic Immunoglobulin Light-Chain Amyloidosis
Clinical Lymphoma and Myeloma, 2006Amyloidosis is a rare disease in which amyloid fibrils compromise organ function and lead to death. Systemic immunoglobulin light-chain amyloidosis, usually caused by free light chains (FLCs) made by clonal plasma cells, is the most frequent type. Hereditary and senile systemic amyloidosis are less frequent types.
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