Pathogenesis and management of high molecular risk myeloproliferative neoplasms
Classical myeloproliferative neoplasms (MPNs) are clonal stem cell disorders characterised by driver mutations that affect the constitutive activation of JAK-signalling. Additional mutations to an MPN-driver occur in a large number of patients and have been shown be associated with disease presenta...
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| Format: | Article |
| Language: | English |
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Ferrata Storti Foundation
2024-12-01
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| Series: | Haematologica |
| Online Access: | https://haematologica.org/article/view/11857 |
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| _version_ | 1850137517825720320 |
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| author | Victoria Y. Ling Florian H. Heidel Megan J. Bywater |
| author_facet | Victoria Y. Ling Florian H. Heidel Megan J. Bywater |
| author_sort | Victoria Y. Ling |
| collection | DOAJ |
| description |
Classical myeloproliferative neoplasms (MPNs) are clonal stem cell disorders characterised by driver mutations that affect the constitutive activation of JAK-signalling. Additional mutations to an MPN-driver occur in a large number of patients and have been shown be associated with disease presentation and progression. In this review, we will outline the current hypotheses regarding how clonal evolution in MPN is thought to occur and the functional mechanisms as to how concomitant somatic mutations (i.e. mutations in genes other than the ‘driver’ genes) contribute to disease progression. We will discuss the definitions of high molecular risk MPN, provide an overview as to how concomitant mutations influence the clinical management of MPN and suggest how this rapidly developing genetic risk stratification can be utilised to improve clinical outcomes.
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| format | Article |
| id | doaj-art-d0c119f5f3df4f2a8b41c05ed9565b6f |
| institution | OA Journals |
| issn | 0390-6078 1592-8721 |
| language | English |
| publishDate | 2024-12-01 |
| publisher | Ferrata Storti Foundation |
| record_format | Article |
| series | Haematologica |
| spelling | doaj-art-d0c119f5f3df4f2a8b41c05ed9565b6f2025-08-20T02:30:49ZengFerrata Storti FoundationHaematologica0390-60781592-87212024-12-01999110.3324/haematol.2023.283987Pathogenesis and management of high molecular risk myeloproliferative neoplasmsVictoria Y. Ling0Florian H. Heidel1Megan J. Bywater2QIMR Berghofer Medical Research Institute, Brisbane, Queensland, Australia; Department of Haematology, Princess Alexandra Hospital, Brisbane, Queensland, Australia; Pathology Queensland, Brisbane, Queensland, Australia; The University of Queensland, Brisbane, QLDHematology, Hemostasis, Oncology and Stem Cell Transplantation, Hannover Medical School (MHH), Hannover, Germany; Leibniz Institute on Aging, Jena, Germany; Cellular Therapy Center (CTC), Hannover Medical School (MHH), HannoverQIMR Berghofer Medical Research Institute, Brisbane, Queensland, Australia; The University of Queensland, Brisbane, QLD Classical myeloproliferative neoplasms (MPNs) are clonal stem cell disorders characterised by driver mutations that affect the constitutive activation of JAK-signalling. Additional mutations to an MPN-driver occur in a large number of patients and have been shown be associated with disease presentation and progression. In this review, we will outline the current hypotheses regarding how clonal evolution in MPN is thought to occur and the functional mechanisms as to how concomitant somatic mutations (i.e. mutations in genes other than the ‘driver’ genes) contribute to disease progression. We will discuss the definitions of high molecular risk MPN, provide an overview as to how concomitant mutations influence the clinical management of MPN and suggest how this rapidly developing genetic risk stratification can be utilised to improve clinical outcomes. https://haematologica.org/article/view/11857 |
| spellingShingle | Victoria Y. Ling Florian H. Heidel Megan J. Bywater Pathogenesis and management of high molecular risk myeloproliferative neoplasms Haematologica |
| title | Pathogenesis and management of high molecular risk myeloproliferative neoplasms |
| title_full | Pathogenesis and management of high molecular risk myeloproliferative neoplasms |
| title_fullStr | Pathogenesis and management of high molecular risk myeloproliferative neoplasms |
| title_full_unstemmed | Pathogenesis and management of high molecular risk myeloproliferative neoplasms |
| title_short | Pathogenesis and management of high molecular risk myeloproliferative neoplasms |
| title_sort | pathogenesis and management of high molecular risk myeloproliferative neoplasms |
| url | https://haematologica.org/article/view/11857 |
| work_keys_str_mv | AT victoriayling pathogenesisandmanagementofhighmolecularriskmyeloproliferativeneoplasms AT florianhheidel pathogenesisandmanagementofhighmolecularriskmyeloproliferativeneoplasms AT meganjbywater pathogenesisandmanagementofhighmolecularriskmyeloproliferativeneoplasms |