July 11, 2024
An article in the American Journal of Hematology reported an in-depth characterization of clinical and molecular differences between primary and secondary myelofibrosis (MF). Researchers also found that two newer prognostic scores, the Mutation-Enhanced International Prognostic Scoring System 70 (MIPSS70) and MIPSS70+ v2.0, stratified risk in patients with secondary MF more accurately than the myelofibrosis secondary to polycythemia vera and essential thrombocythemia prognostic model (MYSEC-PM). These two scores also are the first to account for additional mutations besides SRSF2 status in patients with secondary MF.
Several prognostic scores have been developed for patient risk stratification and appropriate treatment allocation in primary MF. These include the IPSS, Dynamic IPSS (DIPSS), and DIPSS-plus. Studies have highlighted the prognostic significance of molecular alterations in primary MF, particularly mutations in High Molecular Risk (HMR) genes such as ASXL1, EZH2, IDH1/2, SRSF2, and U2AF1. Consequently, these genetic and molecular alterations were incorporated into the latest prognostic scores, including MIPSS70, MIPSS70-plus, and MIPSS70+ v2.0.
“As opposed to primary MF, few studies explored mutations prognosis significance in secondary MF,” explained Matteo Guerra and colleagues. “The MYSEC-PM was specifically developed for secondary MF and displays a more accurate prognostic performance than IPSS and DIPSS. However, no molecular prognostic models accounting for additional mutations in SMF have yet been described.”