Results 41 to 50 of about 258,635 (326)

Clarifying immunoglobulin gene usage in systemic and localized immunoglobulin light-chain amyloidosis by mass spectrometry.

open access: yesBlood, 2017
The goal of this study was to investigate the frequency of use of light-chain variable region (IGVL) genes among patients with systemic (ALS) and localized (ALL) amyloidosis and to assess for associations between IGVL gene usage and organ tropism.
T. Kourelis   +9 more
semanticscholar   +1 more source

Idiotype-specific intravenous immunoglobulin for therapy of immunoglobulin kappa free light chain deficiency

open access: yesHuman Vaccines & Immunotherapeutics, 2019
Deficient antibody production in patients with common variable immunodeficiency (CVID) is accompanied by an inability to produce free light chains (FLCs), particularly kappa (κ) FLC, due to B-cell dysfunction.
Adina Elena Stanciu   +2 more
doaj   +1 more source

Immunoglobulin Light Chain Gene Rearrangements, Receptor Editing and the Development of a Self-Tolerant Antibody Repertoire

open access: yesFrontiers in Immunology, 2018
Discussion of the antibody repertoire usually emphasizes diversity, but a conspicuous feature of the light chain repertoire is its lack of diversity. The diversity of reported allelic variants of germline light chain genes is also limited, even in well ...
A. Collins, C. Watson
semanticscholar   +1 more source

Amyloidosis of the gastrointestinal tract: a 13-year, single-center, referral experience

open access: yesHaematologica, 2013
Amyloidosis of the gastrointestinal tract, with biopsy-proven disease, is rare. We reviewed a series of patients who presented with biopsy-proven gastrointestinal amyloidosis and report their clinical characteristics, treatments, and survival.
Andrew J. Cowan   +7 more
doaj   +1 more source

Venetoclax induced a complete response in a patient with immunoglobulin light chain amyloidosis plateaued on cyclophosphamide, bortezomib and dexamethasone

open access: yesHaematologica, 2018
A 67-year-old male presented with a one-year history of fatigue, cold intolerance, malaise and an unintentional weight loss of ~80 lbs over the course of the preceding 12 months.
N. Leung, S. Thomé, A. Dispenzieri
semanticscholar   +1 more source

Light‐chain amyloid myopathy isolated to skeletal muscles: A case report

open access: yesClinical Case Reports, 2020
Isolated amyloidosis, especially of amyloid light‐chain type, is an infrequent disease. Systemic chemotherapy for light‐chain amyloidosis isolated to skeletal muscles plays a key role to reduce clonal plasma cells producing aberrant immunoglobulin.
Toshihiro Matsukawa   +5 more
doaj   +1 more source

Rapid hematologic responses improve outcomes in patients with very advanced (stage IIIb) cardiac immunoglobulin light chain amyloidosis

open access: yesHaematologica, 2018
Systemic AL amyloidosis (AL) is caused by deposition of misfolded immunoglobulin light chains, leading to potentially catastrophic visceral dysfunction.[1][1] Outcomes are heterogeneous, but cardiac involvement is a key survival predictor.
R. Manwani   +13 more
semanticscholar   +1 more source

Treatment of IgM-associated immunoglobulin light-chain amyloidosis with rituximab-bendamustine.

open access: yesBlood, 2018
TO THE EDITOR: Systemic amyloid light-chain amyloidosis (AL) is characterized by deposition of misfolded immunoglobulin light chains within organs. AL with an immunoglobulin M (IgM) monoclonal protein (IgM-AL) accounts for 5% to 7% of AL and exhibits ...
R. Manwani   +13 more
semanticscholar   +1 more source

Immunoglobulin light chains in medaka (Oryzias latipes) [PDF]

open access: yesImmunogenetics, 2013
The gene segments encoding antibodies have been studied in many capacities and represent some of the best-characterized gene families in traditional animal disease models (mice and humans). To date, multiple immunoglobulin light chain (IgL) isotypes have been found in vertebrates and it is unclear as to which isotypes might be more primordial in nature.
Magadan-Mompo, Susana   +3 more
openaire   +4 more sources

First report of safety and efficacy of daratumumab in 2 cases of advanced immunoglobulin light chain amyloidosis.

open access: yesBlood, 2016
To the editor: Immunoglobulin light chain amyloidosis (AL) with advanced cardiac involvement is characterized by short survival. Outcomes are especially poor for patients who have multiorgan involvement and coexisting multiple myeloma and do not achieve
T. Sher, B. Fenton, A. Akhtar, M. Gertz
semanticscholar   +1 more source

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