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NCCN Flash Updates: NCCN Guidelines Updated for Myeloproliferative Neoplasms

NCCN has published updates to the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), the NCCN Drugs & Biologics Compendium (NCCN Compendium®), and the NCCN Imaging Appropriate Use Criteria (NCCN Imaging AUC™)  for Myeloproliferative Neoplasms. These NCCN Guidelines® are currently available as Version 1.2023.

Link directly to the Updates section of the NCCN Guidelines: Myeloproliferative Neoplasms

MPN-1

  • Bullet 15, modified: Coagulation tests to evaluate for acquired von Willebrand disease (VWD) and /or other...

Footnotes

  • "b", modified: See 2017 WHO Diagnostic Criteria for Primary Myelofibrosis (PMF) International Consensus Classification (ICC) Diagnostic Criteria for Primary Myelofibrosis (MPN-A).
  • "d", modified: Directing the reader to the 2017 WHO ICC Diagnostic Criteria for PV and ET Post-PV MF. (Also for MPN-2A).
  • "g", modified: Patients undergoing high-risk surgical procedures and those with elevated platelet count and/or splenomegaly or unexplained bleeding.

MPN-2

  • Column 2, bullet 3, modified: DIPPS (if recent karyotyping is not available).
  • Column 3, New: Accelerated/blast phase MF is option for myelofibrosis under risk stratification.

MPN-2A

Footnotes

  • "i", modified: The diagnosis of MPN is based on the 20172022 WHO criteria and ICC criteria.requires a combination of clinical, laboratory, cytogenetic, and molecular tests.

MF-1

  • Column 7, bottom pathway, Disease progression, modified: Advanced stage MF/AML Accelerated/blast phase MF (Also for MF-2).

Footnotes

  • "c", modified: Bone marrow aspirate and biopsy with NGS and karyotyping should be performed at diagnosis...
  • "g", modified: ... associated with disease progression in patients with MF PMF, (Also for MF-2).

MF-2

  • Pacritinib:
    • Changed from a category 2A to a category 1, preferred recommendation for higher-risk myelofibrosis with platelets ˂50 x 109/L and not a transplant candidate.
    • Added as a category 2B treatment option for higher-risk myelofibrosis for platelets ≥50 x 109/L and not a transplant candidate for both first-line and subsequent-line.

Footnotes

  • Footnote deleted: Consider pacritinib for patients with platelet counts ≥50 x 109/L with one prior JAK inhibitor.

MF-3

Footnotes

  • Footnote deleted: A clinical trial of luspatercept is preferred (if available).

MF-4

  • Column 1, modified: Accelerated/blast phase MF Disease progression to advanced-phase/AML
  • Column 3, bottom pathway, modified: MF blast phase AML (blasts ≥20% in peripheral blood or bone marrow).

Footnotes

  • Footnote deleted: The WHO classification defines acute leukemia as ≥20% blasts in the marrow or blood. A diagnosis of AML may be made with less than 20% in patients with recurrent cytogenetic abnormalities [eg, t(15;17), t(8;21), t(16;16), inv(16)].
  • "t", modified 3rd sentence: Based on NGS panel results, low intensity /or targeted therapy can be considered.

MF-A 4 of 5

Footnotes

  • "c", modified, Unfavorablekaryotype: complex karyotype or sole or two abnormalities that include trisomy 8, 7/7q-, i(17q), 5/5q-, 12p-, inv(3), or 11q23 rearrangement. any abnormal karyotype other than normal karyotype or sole abnormalities of 20q-, 13q-, +9, chromosome 1 translocation/duplication, or -Y or sex chromosome abnormality other than –Y.

PV-1

  • Ropeginterferon alfa-2b-njft:
    • Column 2, deleted: for upfront polycythemia vera, category 2B, other recommended regimen.
    • Column 7, added: as a preferred cytoreductive therapy option for symptomatic low-risk polycythemia vera.
  • Column 4, modified: disease progression to MF/AML (Also for PV-2, ET-1, ET-2, ET-3 ).
  • Column 6, bottom pathway, modified: Disease progression, modified: Advanced phase MF/AML Accelerated/blast phase MF (Also for PVF-2, ET-1, ET-2, ET-3).

Footnotes

  • Footnote deleted: Bone marrow aspirate and biopsy should be performed to rule out disease progression to myelofibrosis prior to the initiation of cytoreductive therapy. (Also for PV-2).
  • Footnote deleted: The WHO classification defines acute leukemia as ≥20% blasts in the marrow or blood. A diagnosis of AML may be made with less than 20% in patients with recurrent cytogenetic abnormalities [eg, t(15;17), t(8;21), t(16;16), inv(16)], (also for PV-2, ET-1, ET-2, ET-3).
  • "i", modified: Peginterferon alfa-2a can be considered is an option for younger patients or in pregnant patients in need of cytoreductive therapy or in those in need of cytoreductive therapy that defer hydroxyurea or ropeginterferon alfa-2b-njft. (Also for PV-2).

PV-2

  • Ropeginteferon alfa-2b-njft:
    • Column 2, for cytoreductive therapy for high-risk polycythemia vera the preference stratification changed from other recommended regimen to preferred.
  • Column 5, bullet 1, modified: Intolerance or resistance to hydroxyurea, or interferons peginterferon alfa-2a
  • Column 6, modified: Ruxolitinib (category 1 for high-risk PV for hydroxyurea resistance or intolerance).

Footnotes

  • "m", 2nd sentence, New: Ruxolitinib may have activity after inadequate response or loss of response to other agents besides hydroxyurea. See Discussion.

ET-3

Footnotes

  • "j", modified: Bone marrow aspirate and biopsy should be performed to rule out disease progression to MF if clinical/laboratory suspicion of MF. prior to the initiation of cytoreductive therapy.

MPN-A, 1 of 2

  • New page title: 2017 WHO International Consensus Classification (ICC) Diagnostic Criteria for Primary Myelofibrosis.

MPN-C

  • New page title: 2017 WHO ICC Diagnostic Criteria for Polycythemia Vera and Post-PV Myelofibrosis.

MPN-D

  • New page title: 2017 WHO ICC Diagnostic Criteria for Essential Thrombocythemia and Post-ET Myelofibrosis.

MPN-G (1 of 3)

  • Supportive Care for Patients with MPN
    • Myelofibrosis
      • Bullet 9, modified: ... (eg, smoking, diet, exercise, hypertension, diabetes mellitus, lipid management)...

MPN-H (2 of 7)

  • Special Considerations for the use of JAK Inhibitors/Ruxolitinib
    • Bullet 5, new: Major adverse cardiac events: Risk may be increased in people who currently smoke or who previously smoked and patients with other cardiovascular risk factors. Monitor for signs, evaluate and treat promptly. (Also for MPN-H 4 of 7).

MPN-H (4 of 7)

  • Special Considerations for the use of JAK Inhibitors/Fedratinib
    • Bullet 2, modified: Thiamine (vitamin B1) level CBC with platelets...

MPN-I (3 of 4)

  • Special Considerations in the Treatment of Polycythemia Vera and Essential Thrombocythemia.
    • Recommendations for the Management of Standard-Risk Pregnancy
      • Bullet 1, modified: Patients with MPN PV or ET....
    • Definition of High-Risk Pregnancy in MPN PV or ET
      • Pregnancy in the setting ofMPN PV or ET
        • Bullet 2, modified: Previous hemorrhage due to MPN PV or ET

Abbreviations

ABBR-1

  • New section added: Abbreviations

 

For the complete updated versions of the NCCN Guidelines, NCCN Guidelines with NCCN Evidence Blocks™, the NCCN Drugs & Biologics Compendium (NCCN Compendium®), the NCCN Biomarkers Compendium®, the NCCN Chemotherapy Order Templates (NCCN Templates®), the NCCN Radiation Therapy Compendium™, and the NCCN Imaging Appropriate Use Criteria (NCCN Imaging AUC™), please visit NCCN.org.

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