Abstract

Background: There is a paucity of data on ethnic disparities in patients with the classical Philadelphia chromosome-negative myeloproliferative neoplasms (MPNs): polycythaemia vera (PV), essential thrombocythaemia (ET) and primary myelofibrosis (PMF). Methods: This study analysed the demographic data for PV, ET and PMF collected by the New Zealand Cancer Registry (NZCR) between 2010 and 2017. Results: We found that the NZCR capture rates were lower than average international incidence rates for PV and ET, but higher for PMF (0.76, 0.99 and 0.82 per 100,000, respectively). PV patients were older and had worse outcomes than expected, which suggests these patients were reported to the registry at an advanced stage of their disease. Polynesian patients with all MPN subtypes, PV, ET and PMF, were younger than their European counterparts both at the time of diagnosis and death (p < 0.001). Male gender was an independent risk factor for mortality from PV and PMF (hazard ratios (HR) of 1.43 and 1.81, respectively; p < 0.05), and Māori ethnicity was an independent risk factor for mortality from PMF (HR: 2.94; p = 0.006). Conclusions: New Zealand Polynesian patients may have increased genetic predisposition to MPN, thus we advocate for modern genetic testing in this ethnic group to identify the cause. Further work is also required to identify modifiable risk factors for mortality in MPN, in particular those associated with male gender and Māori ethnicity; the results may benefit all patients with MPN.

Highlights

  • Introduction published maps and institutional affilClassical Philadelphia chromosome-negative (Ph-negative) myeloproliferative neoplasms (MPNs) include the clinical entities of polycythaemia vera (PV), essential thrombocythaemia (ET) and primary myelofibrosis (PMF)

  • Between 2010 and 2017, a total of 787 patients were reported to the New Zealand Cancer Registry (NZCR) and diagnosed with one of the three MPNs of interest; 275 (34.9%) had PV, 360 (45.7%) had ET, and

  • This study investigated the incidence and mortality outcomes of patients with classical Ph-negative MPN (PV, ET and PMF) in New Zealand based on the data reported to the NZCR between 2010 and 2017 for PV and ET, and between 2014 and 2017 for PMF

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Summary

Introduction

Classical Philadelphia chromosome-negative (Ph-negative) myeloproliferative neoplasms (MPNs) include the clinical entities of polycythaemia vera (PV), essential thrombocythaemia (ET) and primary myelofibrosis (PMF). A systematic review reported the combined international incidence rates to be 0.84, 1.03, and 0.47 per 100,000 for PV, ET, and PMF, respectively [4]. There was wide variation in incidence rates in the reviewed studies, which included global data from 1935 to 2010. Philadelphia chromosome-negative myeloproliferative neoplasms (MPNs): polycythaemia vera (PV), essential thrombocythaemia (ET) and primary myelofibrosis (PMF). Methods: This study analysed the demographic data for PV, ET and PMF collected by the New Zealand Cancer Registry (NZCR). Results: We found that the NZCR capture rates were lower than average international incidence rates for PV and ET, but higher for PMF (0.76, 0.99 and 0.82 per 100,000, respectively).

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