Hitting the brakes on accelerated and blast-phase myeloproliferative neoplasms: current and emerging concepts Review

  


Authors: Bewersdorf, J. P.; Rampal, R. K.
Review Title: Hitting the brakes on accelerated and blast-phase myeloproliferative neoplasms: current and emerging concepts
Abstract: The BCR-ABL-negative myeloproliferative neoplasms (MPNs) have a variable risk of progressing to accelerated- or blast-phase MPN (MPN-AP/MPN-BP), defined by the presence of 10% to 19% and more than or equal to 20% myeloid blasts in the peripheral blood or bone marrow, respectively. The molecular processes underlying the progression to MPN-AP/MPN-BP are becoming increasingly understood with the acquisition of additional mutations in epigenetic modifiers (eg, ASXL1, EZH2, TET2), TP53, the Ras pathway, or splicing factors (eg, SRSF2, U2AF1), having been described as important steps in this evolutionary process. At least partially driven by the enrichment of these high-risk molecular features, the prognosis of patients with MPN-BP remains inferior to other patients with acute myeloid leukemia, with a median overall survival of 3 to 6 months. Allogeneic hematopoietic cell transplantation remains the only potentially curative therapeutic modality, but only a minority of patients are eligible. In the absence of curative intent, therapeutic strategies or palliative treatment with hypomethylating agents as monotherapy or in combination with ruxolitinib or venetoclax can be considered. Several novel agents are in various stages of clinical development but are not available for routine use at this point, highlighting the need for ongoing research and the prioritization of clinical trial enrollment when feasible. Copyright © 2022 by The American Society of Hematology.
Keywords: myeloproliferative disorders; genetics; mutation; myeloproliferative disorder; leukemia, myeloid, acute; blast cell crisis; blast crisis; acute myeloid leukemia; humans; prognosis; human
Journal Title: Hematology-American Society of Hematology Education Program
Volume: 2022
Issue: 1
ISSN: 1520-4391
Publisher: American Society of Hematology  
Date Published: 2022-12-09
Start Page: 218
End Page: 224
Language: English
DOI: 10.1182/hematology.2022000341
PUBMED: 36485103
PROVIDER: scopus
DOI/URL:

https://www.scopus.com/inward/record.uri?eid=2-s2.0-85143917398&doi=10.1182%2fhematology.2022000341&partnerID=40&md5=38fb10b39667569df166f2210ac8698d
Notes: Export Date: 3 January 2023 -- Source: Scopus
Altmetric
What is Altmetric?
Citation Impact
What is Dimensions Citation Badge?
BMJ Impact Analytics
MSK Authors
  1. Raajit Kumar Rampal
    339 Rampal
  2. Jan Philipp Bewersdorf
    96 Bewersdorf
Related MSK Work