The management of myelofibrosis: A British Society for Haematology Guideline

Abstract

This document represents an update of the British Society for Haematology guideline on Myelofibrosis first published in 2012 and updated in 2015. 1 These guidelines aim to provide healthcare professionals with clear guidance on stratified management for primary myelofibrosis (PMF), as well as postpolycythaemia myelofibrosis (post-PV MF) and postessential thrombocythaemia myelofibrosis (post-ET MF). A separate BSH guideline covers the diagnosis and prognostic evaluation of myelofibrosis and is published alongside this guideline. I N TRODUC T IONMyelofibrosis (MF) encompasses primary myelofibrosis (PMF), postessential thrombocythaemia (ET) MF and

Evaluation of Absolute Neutrophil, Lymphocyte and Platelet Count and Their Ratios as Predictors of Thrombotic Risk in Patients with Prefibrotic and Overt Myelofibrosis

“…Thrombosis imposes a substantial morbidity burden on MF patients [42,43] and mandates the introduction of specific therapies that may further make it difficult to optimize care [44]. Myelodepletive phenotype, low platelet count and developed complications of portal hypertension that are encountered in subsets of MF patients further challenge the delivery of specific primary and secondary prevention measures [45][46][47][48][49][50]. Thrombosis also significantly increases healthcare resource utilization and treatment costs [51], which are already high in patients with MF [52].…”

“…The consequent impairment of functional status associated with thrombotic events may further impact eligibility to some anticancer therapies and push the patient into the sphere of palliative care. Nevertheless, thrombotic risk in MF patients is of secondary interest due to anemia, constitutional symptoms and the risk of death dominating the care environment [2,47,53,54]. As such, there are no official guidelines of prognostic risk factors used for this purpose among MF patients, with notable exception of the original International Prognostic Score of Thrombosis for Essential Thrombocythemia (IPSET-Thrombosis) score, originally developed for ET, being validated among prefibrotic PMF patients [55].…”