Myeloproliferative Neoplasms Clinical Practice Guidelines (NCCN, 2022)

National Comprehensive Cancer Network

These are some of the highlights of the guidelines without analysis or commentary. For more information, go directly to the guidelines by clicking the link in the reference.

September 30, 2022

Clinical practice guidelines on myeloproliferative neoplasms were updated in September 2022 by the National Comprehensive Cancer Network (NCCN).[1]

It is recommended that all patients be assessed at baseline and during treatment using the Myelodysplastic Neoplasm-Symptom Assessment Form Total Symptom Score (MPN-SAF TSS; MPN 10).

The NCCN recommends molecular testing on blood or bone marrow for JAK2 V617F mutations as a component of the initial workup for all patients.

Patients with left-shifted leukocytosis and/or thrombocytosis with basophilia should have fluorescence in situ hybridization (FISH) or multiplex reverse transcription polymerase chain reaction (RT-PCR) on peripheral blood for BCR::ABL1 transcripts to rule out chronic myeloid leukemia (CML).

After confirmation of a myeloproliferative neoplasm diagnosis, a multigene next generation sequencing (NGS) panel that includes JAK2, CALR, and MPL is recommended.

All patients with myelofibrosis should receive referral to specialist care centers with myeloproliferative neoplasm expertise.

Ruxolitinib and fedratinib therapy is recommended for all high-risk myelofibrosis patients with platelet count ≥50 × 109/L who are not transplant candidates. JAK 2 inhibitors should be continued as based on the clinician's judgement if there is a favorable initial response.

Liver function tests and prostate cancer surveillance should be carried out in patients receiving danazol for myelofibrosis-associated anemia.

During the initial workup, molecular testing for acute myeloid leukemia (AML)-associated mutations is recommended for myelofibrosis patients with disease progression to advanced-phase myelofibrosis or transformation to AML.

Myelofibrosis patients with serum erythropoietin (EPO) levels <500 mU/mL should receive treatment with erythropoietin-stimulating agents (ESAs) such as epoetin alfa and darbepoetin alfa.

Enrollment in a clinical trial is recommended for all patients with myelofibrosis requiring treatment.

For more information, please go to Myeloproliferative Disease.

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