Feed

Prefibrotic primary myelofibrosis (pre-PMF) is a subtype of primary myelofibrosis, having only been formally defined as a distinct entity since 2016. Diagnosis, using clinical assessment, peripheral blood analysis, genetic and molecular analysis, and bone marrow biopsy, is needed to distinguish pre-PMF from other myeloproliferative neoplasms, in particular essential thrombocythemia. While the 2022 International Consensus Criteria and World Health Organization diagnostic criteria are used in current clinical practice to aid diagnosis, prognostication for pre-PMF remains challenging. Advances in molecular testing and cytogenetics may enable new risk stratification and prognostic tools to inform treatment decisions for pre-MF. In this review, we aim to give an overview on pre-PMF including the latest updates on diagnostics and prognostic indicators. Furthermore, due to limited reviews on pre-PMF, there still remains no standardized treatment pathway; thus, we summarize the current management advice as well as suggest further trials and areas of research that may improve care in pre-PMF patients.