The National Comprehensive Cancer Network (NCCN) has released updates to their guidelines for myeloproliferative neoplasms, including management suggestions for related disorders and testing options.
Polycythemia vera (PV) and essential thrombocythemia (ET) are new algorithms included in the latest version of the guidelines.
Upon suspicion of myeloproliferative neoplasms, the guidelines suggest using bone marrow cytogenetics (blood, if bone marrow is inaspirable) or molecular testing (blood) for JAK2 V617F mutation. If this latter test is negative, the guidelines recommend testing for CALR and MPL mutations for patients with ET and myelofibrosis and JAK2 Exon 12 mutations for patients with PV.
The guidelines suggest that novel prognostic models are being developed for the risk stratification of post-PV and post-ET myelofibrosis, corresponding to the International Prognostic Scoring System.
Pomalidomide plus prednisone was added as an option for the management of myelofibrosis-associated anemia.
For the management of vascular events, “plateletpheresis may be indicated in patients with ET presenting with acute life-threatening thrombosis or severe bleeding.”—Zachary Bessette