The EMPRESS Study

MEchanisms of hypercoagulability in MyeloProlifeRative nEoplaSmS –The EMPRESS Study

Myeloproliferative neoplasms (MPNs) are chronic bone marrow diseases characterised by clonal proliferation of haematopoietic precursors leading to elevated cell counts in the peripheral blood. MPNs include polycythaemia vera (PV, characterised by peripheral erythrocytosis), essential thrombocytosis (ET, characterised by  thrombocytosis) and myelofibrosis (MF, characterised by megakaryocyte and fibroblast proliferation). Thrombosis is the predominant source of morbidity and mortality for patients. The mechanisms underlying thrombotic risk remain to be fully determined although evidence of abnormal platelet activity has been reported and elevated platelet counts are considered a hallmark of MPNs.

In addition to the role of platelets in thrombosis, platelets are increasingly recognised as being key regulators of other pathological processes. The platelet releasate (PR) refers to the diverse signals (inflammatory mediators & angiogenic factors) which are released by platelets upon activation and include soluble proteins as well as extracellular vesicles (EVs).  EVs are important messengers containing protein signals. 

We hypothesise that the proteomic content of PR is fundamentally altered in patients with MPN.  Characterisation of the PR proteome in conjunction with proteomic analysis of circulating plasma EVs and assessment of mechanisms underlying plasma hypercoagulability in MPN, will reveal novel insights into the pathophysiology of the disease and the associated thrombotic risk.

This work is funded by the Wellcome-Health Research Board Irish Clinical and Academic Training Programme.