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真性红细胞增多症病程中治疗及血细胞计数对生存和血管事件风险影响的回顾性分析

A retrospective analysis of the impact of treatments and blood counts on survival and the risk of vascular events during the course of polycythaemia vera.

作者信息

Enblom-Larsson Anneli, Girodon Francois, Bak Marie, Hersby Ditte, Jooste Valérie, Hasselbalch Hans, Johansson Peter, Andreasson Björn

机构信息

Department of Internal Medicine, Sunderby hospital, Luleå, Sweden.

Service d'hématologie Biologique, CHU de Dijon, Dijon, France.

出版信息

Br J Haematol. 2017 Jun;177(5):800-805. doi: 10.1111/bjh.14625. Epub 2017 May 5.

DOI:10.1111/bjh.14625
PMID:28474342
Abstract

Vascular and non-vascular complications are common in patients with polycythaemia vera. This retrospective study of 217 patients with polycythaemia vera aimed to determine whether blood counts with respect to different treatments influenced the complication rate and survival. We found that 78 (36%) patients suffered from at least one complication during follow-up. Older age and elevated lactate dehydrogenase at diagnosis were found to be risk factors for vascular complications. When the vascular complication occurred, 41% of the patients with a complication had elevated white blood cells (WBC) compared with 20% of patients without a complication (P = 0·042). Patients treated with hydroxycarbamide (HC; also termed hydroxyurea) experienced significantly fewer vascular complications (11%) than patients treated with phlebotomy only (27%) (P = 0·013). We also found a survival advantage for patients treated with HC, when adjusted for age, gender and time period of diagnosis (Hazard ratio for phlebotomy-treated patients compared to HC-treated patients at 5 years was 2·42, 95% confidence interval 1·03-5·72, P = 0·043). Concerning survival and vascular complications, HC-treated patients who needed at least one phlebotomy per year were not significantly different from HC-treated patients with a low phlebotomy requirement. We conclude that complementary phlebotomy in HC-treated patients in order to maintain the haematocrit, is safe.

摘要

真性红细胞增多症患者常见血管和非血管并发症。这项对217例真性红细胞增多症患者的回顾性研究旨在确定不同治疗方式下的血细胞计数是否会影响并发症发生率和生存率。我们发现,78例(36%)患者在随访期间至少出现了一种并发症。研究发现,年龄较大以及诊断时乳酸脱氢酶升高是血管并发症的危险因素。发生血管并发症时,出现并发症的患者中有41%白细胞(WBC)升高,而无并发症的患者中这一比例为20%(P = 0·042)。接受羟基脲(HC;也称为羟基尿素)治疗的患者出现血管并发症的比例(11%)明显低于仅接受放血治疗的患者(27%)(P =

0·013)。我们还发现,在对年龄、性别和诊断时间段进行调整后,接受HC治疗的患者具有生存优势(与接受HC治疗的患者相比,放血治疗患者在第5年的风险比为2·42,95%置信区间为1·03 - 5·72,P = 0·043)。关于生存和血管并发症,每年至少需要进行一次放血的HC治疗患者与放血需求较低的HC治疗患者没有显著差异。我们得出结论,为维持血细胞比容,对接受HC治疗的患者进行辅助放血是安全的。

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The very-long-term course of polycythaemia: a complement to the previously published data of the Polycythaemia Vera Study Group.
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