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骨髓纤维化患者的生存和血管并发症风险:来自瑞典 MPN 小组的一项基于人群的研究。

Survival and risk of vascular complications in myelofibrosis-A population-based study from the Swedish MPN group.

机构信息

Department of Medicine, Kalmar County Hospital, Kalmar, Sweden.

Faculty of Medicine, Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden.

出版信息

Eur J Haematol. 2022 Oct;109(4):336-342. doi: 10.1111/ejh.13813. Epub 2022 Jun 24.

Abstract

OBJECTIVE

To gain knowledge of underlying risk factors for vascular complications and their impact on life expectancy in myelofibrosis.

METHODS

From a cohort of 392 myelofibrosis patients registered in the Swedish MPN registry 58 patients with vascular complications during follow-up were identified. Patients with vascular complications were compared with both 1:1 matched controls and the entire myelofibrosis cohort to explore potential risk factors for vascular complications and their impact on survival.

RESULTS

Incidence of vascular complications was 2.8 events per 100 patient-years and the majority of complications were thrombotic. Patients with complications were significantly older and had lower hemoglobin when compared to the entire cohort. In the case-control analysis, no significant risk factor differences were observed. The major cause of death was vascular complications and median survival was significantly impaired in patients with vascular complications (48 months) compared to controls (92 months). Inferior survival in patients with vascular complications was found to be dependent on IPSS risk category in a Cox regression model.

CONCLUSION

Vascular complications have a considerable impact on survival in MF. At diagnosis, risk assessment by IPSS does not only predict survival but is also associated with the risk of vascular complications.

摘要

目的

了解骨髓纤维化患者血管并发症的潜在风险因素及其对预期寿命的影响。

方法

在瑞典 MPN 登记处登记的 392 例骨髓纤维化患者中,有 58 例在随访期间发生血管并发症。将发生血管并发症的患者与 1:1 匹配的对照组和整个骨髓纤维化队列进行比较,以探讨血管并发症的潜在风险因素及其对生存的影响。

结果

血管并发症的发生率为每 100 名患者年 2.8 例,大多数并发症为血栓形成。与整个队列相比,发生并发症的患者年龄明显更大,血红蛋白水平更低。在病例对照分析中,未观察到显著的风险因素差异。主要死亡原因为血管并发症,与对照组(92 个月)相比,有血管并发症的患者的中位生存时间显著缩短(48 个月)。Cox 回归模型显示,血管并发症患者的生存预后较差与 IPSS 风险类别相关。

结论

血管并发症对 MF 的生存有相当大的影响。在诊断时,IPSS 风险评估不仅预测生存,而且与血管并发症的风险相关。

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