免疫球蛋白轻链
淀粉样变性
淀粉样变性
多发性骨髓瘤
克隆(Java方法)
等离子体电池
生物
不确定意义的单克隆抗体病
抗体
单克隆
淀粉样蛋白(真菌学)
浆细胞失调
骨髓
分子生物学
病理
单克隆抗体
基因
免疫学
医学
遗传学
作者
Kenji Kimura,Shokichi Tsukamoto,Kanji Miyazaki,Chika Kawajiri-Manako,Arata Ishii,Bahityar Rahmutulla,Masaki Fukuyo,Nagisa Oshima-Hasegawa,Shio Mitsukawa,Yusuke Takeda,Naoya Mimura,Masahiro Takeuchi,Chikako Ohwada,Tohru Iseki,Keisuke Matsusaka,Masashi Sanada,Koutaro Yokote,Atsushi Kaneda,Tadao Ishida,Kenshi Suzuki,Chiaki Nakaseko,Emiko Sakaida
标识
DOI:10.1016/j.exphem.2021.08.001
摘要
Amyloid light-chain (AL) amyloidosis is caused by deposition of abnormally folded clonal immunoglobulin (Ig) light chains made by malignant plasma cells in the bone marrow (BM), leading to multiorgan dysfunction. However, little is known of the factors that regulate the organ tropism of amyloid deposition in this disease. We aimed to identify the clonal composition of Igλ light-chain variable region (IGLV) genes in BM cells in patients with AL amyloidosis using next-generation sequencing. Based on our definition of the clonal IGLV rearrangement (dominant clone >2.5%, dominant cluster >5%), we identified clonal IGLV in 33 of 38 patients with AL amyloidosis (86.8%), 6 of 9 with monoclonal gammopathy of undetermined significance (67%), and 7 of 7 with multiple myeloma (100%). The clones in AL amyloidosis were significantly smaller than those in multiple myeloma (p < 0.01) but comparable to those in monoclonal gammopathy of undetermined significance. Importantly, in patients with AL amyloidosis, the difference in involved and uninvolved free light chains was not correlated with the clonal size of BM plasma cells in our repertoire analysis using NGS. In summary, the clonal composition of IGLV genes in the BM was successfully identified in most patients with AL amyloidosis using NGS. The clonal size of plasma cells in the BM is small, and small malignant clones of plasma cells may secrete free light chi and cause light chain depositions in AL amyloidosis.
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