Rie Nakamoto-Matsubara, Valentina Nardi, Nora Horick, Tsuyoshi Fukushima, Ryan S. Han, Rajib Shome, Kiyosumi Ochi, Cristina Panaroni, Keertik Fulzele, Farah Rexha, Andrew R. Branagan, Diana Cirstea, Andrew J. Yee, David T. Scadden, Noopur S. Raje
{"title":"多发性骨髓瘤髓外病变的临床结果与分子特征的整合","authors":"Rie Nakamoto-Matsubara, Valentina Nardi, Nora Horick, Tsuyoshi Fukushima, Ryan S. Han, Rajib Shome, Kiyosumi Ochi, Cristina Panaroni, Keertik Fulzele, Farah Rexha, Andrew R. Branagan, Diana Cirstea, Andrew J. Yee, David T. Scadden, Noopur S. Raje","doi":"10.1038/s41408-024-01190-9","DOIUrl":null,"url":null,"abstract":"<p>Multiple myeloma (MM) remains incurable despite novel therapeutics. A major contributor to the development of relapsed/refractory and resistant MM is extraosseous extramedullary disease (EMD), whose molecular biology is still not fully understood. We analyzed 528 MM patients who presented to our institution between 2014 and 2021 and who had undergone molecular testing. We defined EMD as organ plasmacytoma distinct from bones and evaluated patients for the development of EMD with the goal of defining their molecular characteristics. Here, we show that <i>RAS/BRAF</i> mutations are likely essential for the development of EMD. Our results also indicate that the underlying reason for the negative outcomes in patients with poor prognostic factors such as duplication 1q and deletion 17p is largely due to the development of EMD. However, the presence of <i>TP53</i> mutation remains a poor prognostic factor regardless of EMD development. Furthermore, mutation sites of <i>TP53</i> were different between EMD versus non-EMD patients, with gain-of-function mutations enriched in patients with EMD. Our data highlights distinct molecular abnormalities in patients with EMD and provides potential mechanistic insights for novel therapeutic targets for the future.</p>","PeriodicalId":8989,"journal":{"name":"Blood Cancer Journal","volume":"280 1","pages":""},"PeriodicalIF":12.9000,"publicationDate":"2024-12-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Integration of clinical outcomes and molecular features in extramedullary disease in multiple myeloma\",\"authors\":\"Rie Nakamoto-Matsubara, Valentina Nardi, Nora Horick, Tsuyoshi Fukushima, Ryan S. Han, Rajib Shome, Kiyosumi Ochi, Cristina Panaroni, Keertik Fulzele, Farah Rexha, Andrew R. Branagan, Diana Cirstea, Andrew J. Yee, David T. Scadden, Noopur S. Raje\",\"doi\":\"10.1038/s41408-024-01190-9\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p>Multiple myeloma (MM) remains incurable despite novel therapeutics. A major contributor to the development of relapsed/refractory and resistant MM is extraosseous extramedullary disease (EMD), whose molecular biology is still not fully understood. We analyzed 528 MM patients who presented to our institution between 2014 and 2021 and who had undergone molecular testing. We defined EMD as organ plasmacytoma distinct from bones and evaluated patients for the development of EMD with the goal of defining their molecular characteristics. Here, we show that <i>RAS/BRAF</i> mutations are likely essential for the development of EMD. Our results also indicate that the underlying reason for the negative outcomes in patients with poor prognostic factors such as duplication 1q and deletion 17p is largely due to the development of EMD. However, the presence of <i>TP53</i> mutation remains a poor prognostic factor regardless of EMD development. Furthermore, mutation sites of <i>TP53</i> were different between EMD versus non-EMD patients, with gain-of-function mutations enriched in patients with EMD. Our data highlights distinct molecular abnormalities in patients with EMD and provides potential mechanistic insights for novel therapeutic targets for the future.</p>\",\"PeriodicalId\":8989,\"journal\":{\"name\":\"Blood Cancer Journal\",\"volume\":\"280 1\",\"pages\":\"\"},\"PeriodicalIF\":12.9000,\"publicationDate\":\"2024-12-23\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Blood Cancer Journal\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.1038/s41408-024-01190-9\",\"RegionNum\":1,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q1\",\"JCRName\":\"HEMATOLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Blood Cancer Journal","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1038/s41408-024-01190-9","RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"HEMATOLOGY","Score":null,"Total":0}
Integration of clinical outcomes and molecular features in extramedullary disease in multiple myeloma
Multiple myeloma (MM) remains incurable despite novel therapeutics. A major contributor to the development of relapsed/refractory and resistant MM is extraosseous extramedullary disease (EMD), whose molecular biology is still not fully understood. We analyzed 528 MM patients who presented to our institution between 2014 and 2021 and who had undergone molecular testing. We defined EMD as organ plasmacytoma distinct from bones and evaluated patients for the development of EMD with the goal of defining their molecular characteristics. Here, we show that RAS/BRAF mutations are likely essential for the development of EMD. Our results also indicate that the underlying reason for the negative outcomes in patients with poor prognostic factors such as duplication 1q and deletion 17p is largely due to the development of EMD. However, the presence of TP53 mutation remains a poor prognostic factor regardless of EMD development. Furthermore, mutation sites of TP53 were different between EMD versus non-EMD patients, with gain-of-function mutations enriched in patients with EMD. Our data highlights distinct molecular abnormalities in patients with EMD and provides potential mechanistic insights for novel therapeutic targets for the future.
期刊介绍:
Blood Cancer Journal is dedicated to publishing high-quality articles related to hematologic malignancies and related disorders. The journal welcomes submissions of original research, reviews, guidelines, and letters that are deemed to have a significant impact in the field. While the journal covers a wide range of topics, it particularly focuses on areas such as:
Preclinical studies of new compounds, especially those that provide mechanistic insights
Clinical trials and observations
Reviews related to new drugs and current management of hematologic malignancies
Novel observations related to new mutations, molecular pathways, and tumor genomics
Blood Cancer Journal offers a forum for expedited publication of novel observations regarding new mutations or altered pathways.