News in the treatment of AL amyloidosis

被引:1
|
作者
Jaccard, Arnaud [1 ,2 ]
Mohty, Dania [1 ,3 ]
Desport, Estelle [1 ,4 ]
Bridoux, Frank [1 ,4 ]
机构
[1] CHU Limoges, Hop Dupuytren, Ctr Natl Reference Amylose AL & Autres Malad Depo, 2 Ave Martin Luther King, F-87042 Limoges, France
[2] CHU Limoges, Hop Dupuytren, Serv Hematol & Therapie Cellulaire, F-87042 Limoges, France
[3] CHU Limoges, Serv Cardiol, Limoges, France
[4] CHU Poitiers, Serv Nephrol Hemodialyse & Transplantat Renale, Poitiers, France
来源
HEMATOLOGIE | 2012年 / 18卷 / 02期
关键词
AL amyloidosis; M-Dex; bortezomib; cardiac biomarker;
D O I
10.1684/hma.2012.0692
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Systemic AL amyloidosis is a severe illness. Its prognosis depends on the extent of amyloid deposits and the importance of cardiac involvement. Without effective treatment the median survival is about 12 months [1], but the notion that the formation of amyloid deposits is an irreversible process is false. There is a balance between formation and removal of deposits, that varies considerably among patients and according to the organs. The removal or reduction of the monoclonal light chain serum concentration of the origin of deposits may tip the balance and be accompanied by a progressive elimination of deposits. The degree of reduction in the production of monoclonal light chains necessary for tissue removal, varies between patients and depends on the turnover of amyloid deposits. If the treatment is effective, clinical improvement can sometimes be rapid and dramatic, or more often occur after many months.
引用
收藏
页码:116 / 123
页数:8
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