AL淀粉样变性的治疗:走向个体化和克隆导向治疗

IF 0.9 Q4 HEMATOLOGY
Hemato Pub Date : 2021-12-07 DOI:10.3390/hemato2040050
U. Hegenbart, M. Raab, S. Schönland
{"title":"AL淀粉样变性的治疗:走向个体化和克隆导向治疗","authors":"U. Hegenbart, M. Raab, S. Schönland","doi":"10.3390/hemato2040050","DOIUrl":null,"url":null,"abstract":"Systemic amyloid light chain (AL) amyloidosis is a rare protein deposition disease caused by a clonal B cell disorder of the bone marrow. The underlying diseases can be plasma cell disorders (monoclonal gammopathy of clinical significance, smoldering or symptomatic myeloma) or B cell non-Hodgkin’s lymphoma (e.g., Waldenstrom’s disease or marginal zone lymphoma) with secretory activity. It is crucial to characterize the underlying disease very precisely as the treatment of AL amyloidosis is directed against the (often small) B cell clone. Finally, the detection of cytogenetic aberrations of the plasma cell clone will likely play an important role for choosing an effective drug in the near future.","PeriodicalId":93705,"journal":{"name":"Hemato","volume":" ","pages":""},"PeriodicalIF":0.9000,"publicationDate":"2021-12-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Treatment in AL Amyloidosis: Moving towards Individualized and Clone-Directed Therapy\",\"authors\":\"U. Hegenbart, M. Raab, S. Schönland\",\"doi\":\"10.3390/hemato2040050\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"Systemic amyloid light chain (AL) amyloidosis is a rare protein deposition disease caused by a clonal B cell disorder of the bone marrow. The underlying diseases can be plasma cell disorders (monoclonal gammopathy of clinical significance, smoldering or symptomatic myeloma) or B cell non-Hodgkin’s lymphoma (e.g., Waldenstrom’s disease or marginal zone lymphoma) with secretory activity. It is crucial to characterize the underlying disease very precisely as the treatment of AL amyloidosis is directed against the (often small) B cell clone. Finally, the detection of cytogenetic aberrations of the plasma cell clone will likely play an important role for choosing an effective drug in the near future.\",\"PeriodicalId\":93705,\"journal\":{\"name\":\"Hemato\",\"volume\":\" \",\"pages\":\"\"},\"PeriodicalIF\":0.9000,\"publicationDate\":\"2021-12-07\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Hemato\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.3390/hemato2040050\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q4\",\"JCRName\":\"HEMATOLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Hemato","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.3390/hemato2040050","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"HEMATOLOGY","Score":null,"Total":0}
引用次数: 0

摘要

系统性淀粉样蛋白轻链(AL)淀粉样变性是一种罕见的由骨髓克隆性B细胞紊乱引起的蛋白质沉积疾病。基础疾病可以是浆细胞疾病(有临床意义的单克隆伽玛病、阴燃或症状性骨髓瘤)或具有分泌活性的B细胞非霍奇金淋巴瘤(如瓦尔登斯特罗姆病或边缘带淋巴瘤)。由于AL淀粉样变的治疗是针对(通常是小的)B细胞克隆的,因此非常精确地描述潜在疾病的特征是至关重要的。最后,在不久的将来,检测浆细胞克隆的细胞遗传学畸变可能会在选择有效药物方面发挥重要作用。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Treatment in AL Amyloidosis: Moving towards Individualized and Clone-Directed Therapy
Systemic amyloid light chain (AL) amyloidosis is a rare protein deposition disease caused by a clonal B cell disorder of the bone marrow. The underlying diseases can be plasma cell disorders (monoclonal gammopathy of clinical significance, smoldering or symptomatic myeloma) or B cell non-Hodgkin’s lymphoma (e.g., Waldenstrom’s disease or marginal zone lymphoma) with secretory activity. It is crucial to characterize the underlying disease very precisely as the treatment of AL amyloidosis is directed against the (often small) B cell clone. Finally, the detection of cytogenetic aberrations of the plasma cell clone will likely play an important role for choosing an effective drug in the near future.
求助全文
通过发布文献求助,成功后即可免费获取论文全文。 去求助
来源期刊
CiteScore
1.30
自引率
0.00%
发文量
0
审稿时长
11 weeks
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
确定
请完成安全验证×
copy
已复制链接
快去分享给好友吧!
我知道了
右上角分享
点击右上角分享
0
联系我们:info@booksci.cn Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。 Copyright © 2023 布克学术 All rights reserved.
京ICP备2023020795号-1
ghs 京公网安备 11010802042870号
Book学术文献互助
Book学术文献互助群
群 号:481959085
Book学术官方微信