scholarly journals Localized Immunoglobulin Light-Chain Amyloidosis of the Ulnar Nerve

2021 ◽  
pp. 305-311
Author(s):  
Shinsuke Morisaki ◽  
Shinji Tsuchida ◽  
Eiichi Konishi ◽  
Nagaaki Katoh ◽  
Yusuke Takahashi ◽  
...  
Keyword(s):  
Ulnar Nerve ◽  
Light Chain ◽  
Histopathological Examination ◽  
Systemic Amyloidosis ◽  
Immunoglobulin Light Chain ◽  
Multisystem Disease ◽  
Light Chain Amyloidosis ◽  
Aged Woman ◽  
Ulnar Nerve Palsy ◽  
Immunoglobulin Light Chain Amyloidosis

Amyloidosis is a disorder caused by extracellular tissue deposition of insoluble fibrils. Amyloidosis can be divided into systemic or localized disease. Primary systemic amyloidosis is a multisystem disease caused by the deposition of amyloid in various tissues. Localized amyloidosis has different characteristics than those of systemic amyloidosis. In this paper, we present the case of a middle-aged woman who presented with worsening ulnar nerve palsy. Electrophysiological examination and MRI indicated a tumor surrounding the ulnar nerve in the forearm. However, the operative findings revealed that ulnar nerve fascicles were replaced with a yellow tissue, which was diagnosed as amyloid light-chain λ amyloidosis, based on histopathological examination. Systemic amyloidosis was ruled out after the screening examinations. This paper is the first report of the ulnar nerve as the sole site of localized immunoglobulin light-chain amyloidosis manifestation.

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.

Decrease of B-type natriuretic peptide to less than 200 pg/mL predicts longer survival in cardiac immunoglobulin light chain amyloidosis

2015 ◽  
Vol 102 (2) ◽  
pp. 200-204 ◽  
Author(s):  
Kazuya Ishiguro ◽  
Toshiaki Hayashi ◽  
Tetsuyuki Igarashi ◽  
Yumiko Maruyama ◽  
Hiroshi Ikeda ◽  
...  
Keyword(s):  
Natriuretic Peptide ◽  
Light Chain ◽  
Immunoglobulin Light Chain ◽  
Light Chain Amyloidosis ◽  
Immunoglobulin Light Chain Amyloidosis
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