Integrating AIPSS-MF and molecular predictors: A comparative analysis of prognostic models for myelofibrosis

Abstract

[EN]Myelofibrosis (MF) is a chronic myeloproliferative neoplasm that can manifest as a primary condition (primary myelofibrosis [PMF]) or after progression from a polycythemia vera or essential thrombocythemia (secondary myelofibrosis [SMF]). The aberrant activation of the JAK‐STAT pathway is central to MF pathogenesis which is caused by driver mutations in JAK2, CALR, and MPL genes. These mutations, along with additional somatic variants that mainly impact epigenetic modifiers or spliceosome components, shape the clinical features of the disease. Although the median overall survival (OS) is around 6 years, the clinical course of MF is heterogeneous. The only curative strategy, allogeneic hematopoietic cell transplantation, carries a significant risk of early mortality.2 It is therefore critical to accurately assess transplantation risk and estimate survival with medical therapies to determine the most appropriate treatment approach for each individual.