scholarly journals Venetoclax induces profound and sustained responses in patients with relapsed/refractory light‐chain amyloidosis

2021 ◽  
Vol 193 (3) ◽  
pp. 674-677
Author(s):  
Hélène Pasquer ◽  
Karim Belhadj ◽  
Jehan Dupuis ◽  
Sylvia Oghina ◽  
Antoine Galat ◽  
...  
Keyword(s):  
Light Chain ◽  
Light Chain Amyloidosis ◽  
Sustained Responses

Prognostic stratification and treatment of cardiac light chain amyloidosis: A narrow path in the jungle

2014 ◽  
Vol 33 (2) ◽  
pp. 136-138 ◽  
Author(s):  
Luciano Potena ◽  
Candida Cristina Quarta ◽  
Francesco Grigioni ◽  
Claudio Rapezzi
Keyword(s):  
Light Chain ◽  
Light Chain Amyloidosis ◽  
Prognostic Stratification

scholarly journals Genetic pathogenesis of immunoglobulin light chain amyloidosis: basic characteristics and clinical applications

2021 ◽  
Vol 10 (1) ◽  
Author(s):  
Linchun Xu ◽  
Yongzhong Su
Keyword(s):  
Plasma Cell ◽  
Light Chain ◽  
Amyloid Fibril ◽  
Treatment Modalities ◽  
Driver Gene ◽  
Immunoglobulin Light Chain ◽  
Disease Evolution ◽  
Genetic Aberrations ◽  
Light Chain Amyloidosis ◽  
Immunoglobulin Light Chain Amyloidosis

AbstractImmunoglobulin light chain amyloidosis (AL) is an indolent plasma cell disorder characterized by free immunoglobulin light chain (FLC) misfolding and amyloid fibril deposition. The cytogenetic pattern of AL shows profound similarity with that of other plasma cell disorders but harbors distinct features. AL can be classified into two primary subtypes: non-hyperdiploidy and hyperdiploidy. Non-hyperdiploidy usually involves immunoglobulin heavy chain translocations, and t(11;14) is the hallmark of this disease. T(11;14) is associated with low plasma cell count but high FLC level and displays distinct response outcomes to different treatment modalities. Hyperdiploidy is associated with plasmacytosis and subclone formation, and it generally confers a neutral or inferior prognostic outcome. Other chromosome abnormalities and driver gene mutations are considered as secondary cytogenetic aberrations that occur during disease evolution. These genetic aberrations contribute to the proliferation of plasma cells, which secrete excess FLC for amyloid deposition. Other genetic factors, such as specific usage of immunoglobulin light chain germline genes and light chain somatic mutations, also play an essential role in amyloid fibril deposition in AL. This paper will propose a framework of AL classification based on genetic aberrations and discuss the amyloid formation of AL from a genetic aspect.

Severe intrahepatic cholestasis as the initial manifestation of light chain amyloidosis

2021 ◽  
Vol 44 (1) ◽  
pp. 29-31
Author(s):  
Diego Martínez-Acitores de la Mata ◽  
Silvia Bravo Meléndez ◽  
Candela Ceballos Bolaños ◽  
Irene Amat Villegas ◽  
María Carmen Mateos Rodriguez ◽  
...  
Keyword(s):  
Light Chain ◽  
Intrahepatic Cholestasis ◽  
Initial Manifestation ◽  
Light Chain Amyloidosis

scholarly journals Improvements in Cardiac Biomarkers are Associated with Better Health-Related Quality of Life in Patients with Light Chain Amyloidosis

2017 ◽  
Vol 23 (8) ◽  
pp. S114
Author(s):  
Kristen McCausland ◽  
Spencer D. Guthrie ◽  
Tiffany Quock ◽  
Miyo Yokota ◽  
Martha Bayliss ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Light Chain ◽  
Cardiac Biomarkers ◽  
Health Related Quality ◽  
Light Chain Amyloidosis ◽  
Related Quality ◽  
Health Related
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