Updates on accelerated and blast phase myeloproliferative neoplasms: Are we making progress?

• Published in: British journal of haematology Vol. 203; no. 2; pp. 169 - 181
• Main Authors: Mahdi, Dina, Spiers, Jessica, Rampotas, Alexandros, Polverelli, Nicola, McLornan, Donal P
• Format: Journal Article
• Language: English
• Published: England Blackwell Publishing Ltd 01.10.2023
• Subjects: Allografts; Hematology; Hematopoietic stem cells; AML; accelerated phase; blast phase; polycythaemia vera; MPN; JAK2; essential thrombocythaemia
• ISSN: 0007-1048; 1365-2141
• DOI: 10.1111/bjh.19010

Management approaches for accelerated and blast phase myeloproliferative neoplasms remain challenging for clinicians and patients alike. Despite many therapeutic advances, outcomes for those patients who are not allogeneic haematopoietic cell transplant eligible remain, in general, very poor. Estimated survival rates for such blast phase patients is frequently reported as less than 6 months. No specific immunological, genomic or clinicopathological signature currently exists that accurately predicts the risk and timing of transformation, which frequently induces a high degree of anxiety among patients and clinicians alike. Within this review article, we provide an up-to-date summary of current understanding of the underlying pathogenesis of accelerated and blast phase disease and discuss current therapeutic approaches and realistic outcomes. Finally, we discuss how the horizon may look with the introduction of more novel agents into the clinical arena.