SOHO State of the Art Updates and Next Questions | Accelerated Phase of MPN: What It Is and What to Do About It

Anand A Patel; Olatoyosi Odenike

doi:10.1016/j.clml.2023.01.011

SOHO State of the Art Updates and Next Questions | Accelerated Phase of MPN: What It Is and What to Do About It

Clin Lymphoma Myeloma Leuk. 2023 May;23(5):303-309. doi: 10.1016/j.clml.2023.01.011. Epub 2023 Jan 25.

Authors

Anand A Patel¹, Olatoyosi Odenike²

Affiliations

¹ Section of Hematology/Oncology, Department of Medicine, University of Chicago, Chicago, IL.
² Section of Hematology/Oncology, Department of Medicine, University of Chicago, Chicago, IL. Electronic address: todenike@medicine.bsd.uchicago.edu.

PMID: 36907766
DOI: 10.1016/j.clml.2023.01.011

Abstract

Progression of Philadelphia-chromosome negative myeloproliferative neoplasms (MPNs) to the accelerated phase (AP) or blast phase (BP) is associated with poor outcomes. As our understanding of the molecular drivers of MPN progression has grown, there has been increasing investigation into the use of novel targeted approaches in the treatment of these diseases. In this review we summarize the clinical and molecular risk factors for progression to MPN-AP/BP followed by discussion of treatment approach. We also highlight outcomes using conventional approaches such as intensive chemotherapy and hypomethylating agents along with considerations around allogeneic hematopoietic stem cell transplant. We then focus on novel targeted approaches in MPN-AP/BP including venetoclax-based regimens, IDH inhibition, and ongoing prospective clinical trials.

Keywords: BCL2 inhibition; Blast-phase; IDH inhibition; Targeted therapy; hypomethylating agents.

Publication types

Review
Research Support, Non-U.S. Gov't

MeSH terms

Blast Crisis / drug therapy
Hematopoietic Stem Cell Transplantation*
Humans
Myeloproliferative Disorders* / diagnosis
Myeloproliferative Disorders* / drug therapy
Myeloproliferative Disorders* / genetics
Prospective Studies
Risk Factors