{"title":"Myeloproliferative neoplasm harboring both monosomy 7 and an ALK/ROS1 fusion gene: Proposal for a new disease entity","authors":"Hideki Yoshida, Shinya Osone, Madoka Konishi, Seiji Tanaka, Tohru Inaba, Toshihiko Imamura, Tomoko Iehara","doi":"10.1002/jha2.1071","DOIUrl":null,"url":null,"abstract":"<div>\n \n \n <section>\n \n <h3> Introduction</h3>\n \n <p>We present a case of a 9-year-old girl diagnosed with a myeloproliferative neoplasm (MPN) harboring both monosomy 7 and an ALK/ROS1 fusion gene.</p>\n </section>\n \n <section>\n \n <h3> Case presentation</h3>\n \n <p>The neoplasm was resistant to conventional AML chemotherapy and required hematopoietic cell transplantation (HCT) to achieve remission.</p>\n </section>\n \n <section>\n \n <h3> Discussion</h3>\n \n <p>MPNs with monosomy 7 and ALK/ROS1 fusions occur in a wide age range of children and adults, and require HCT for long-term remission. Furthermore, these cases can be responsive to ALK inhibitors.</p>\n </section>\n \n <section>\n \n <h3> Conclusion</h3>\n \n <p>This report underscores the potential need to reclassify such MPNs as a distinct entity, which has unique therapeutic implications.</p>\n </section>\n </div>","PeriodicalId":72883,"journal":{"name":"EJHaem","volume":"6 1","pages":""},"PeriodicalIF":0.0000,"publicationDate":"2024-12-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11756977/pdf/","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"EJHaem","FirstCategoryId":"1085","ListUrlMain":"https://onlinelibrary.wiley.com/doi/10.1002/jha2.1071","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
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Abstract
Introduction
We present a case of a 9-year-old girl diagnosed with a myeloproliferative neoplasm (MPN) harboring both monosomy 7 and an ALK/ROS1 fusion gene.
Case presentation
The neoplasm was resistant to conventional AML chemotherapy and required hematopoietic cell transplantation (HCT) to achieve remission.
Discussion
MPNs with monosomy 7 and ALK/ROS1 fusions occur in a wide age range of children and adults, and require HCT for long-term remission. Furthermore, these cases can be responsive to ALK inhibitors.
Conclusion
This report underscores the potential need to reclassify such MPNs as a distinct entity, which has unique therapeutic implications.