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2010 年至 2017 年新西兰骨髓增殖性肿瘤的流行病学:来自新西兰癌症登记处的见解。

The Epidemiology of Myeloproliferative Neoplasms in New Zealand between 2010 and 2017: Insights from the New Zealand Cancer Registry.

机构信息

Blood and Cancer Biology Laboratory, Department of Molecular Medicine & Pathology, School of Medical Sciences, The University of Auckland, Auckland 1023, New Zealand.

Department of Pathology and Laboratory Medicine, Auckland City Hospital, Auckland 1023, New Zealand.

出版信息

Curr Oncol. 2021 Apr 18;28(2):1544-1557. doi: 10.3390/curroncol28020146.

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 ( < 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; < 0.05), and Māori ethnicity was an independent risk factor for mortality from PMF (HR: 2.94; = 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.

摘要

背景

在经典费城染色体阴性骨髓增殖性肿瘤(MPN)患者中,种族差异的数据很少:真性红细胞增多症(PV)、特发性血小板增多症(ET)和原发性骨髓纤维化(PMF)。

方法

本研究分析了新西兰癌症登记处(NZCR)在 2010 年至 2017 年期间收集的 PV、ET 和 PMF 的人口统计学数据。

结果

我们发现,NZCR 的捕获率低于 PV 和 ET 的国际平均发病率,但 PMF 的捕获率较高(分别为 0.76、0.99 和 0.82/100,000)。PV 患者年龄较大,预后较差,这表明这些患者在疾病的晚期向登记处报告。所有 MPN 亚型、PV、ET 和 PMF 的波利尼西亚患者在诊断和死亡时都比欧洲患者年轻(<0.001)。男性是 PV 和 PMF 死亡的独立危险因素(HR 分别为 1.43 和 1.81;<0.05),毛利族裔是 PMF 死亡的独立危险因素(HR:2.94;=0.006)。

结论

新西兰波利尼西亚患者可能对 MPN 有更高的遗传易感性,因此我们主张在该族裔中进行现代基因检测以确定病因。还需要进一步研究确定 MPN 死亡率的可改变危险因素,特别是与男性和毛利族裔相关的危险因素;结果可能使所有 MPN 患者受益。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d36c/8167767/6dafa3c256f4/curroncol-28-00146-g001.jpg

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