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[匈牙利费城阴性慢性骨髓增殖性肿瘤登记处的审核。原发性血小板增多症患者的评估]

[An audit of the Hungarian Philadelphia negative chronic myeloproliferative neoplasia registry. Evaluation of the essential thrombocythaemic patients].

作者信息

Dombi Péter, Illés Árpád, Demeter Judit, Homor Lajos, Simon Zsófia, Udvardy Miklós, Karádi Éva, Kellner Ádám, Egyed Miklós

机构信息

Szent Borbála Kórház Tatabánya, Dózsa Gy. út 77., 2800.

Hematológiai Tanszék, Debreceni Egyetem, Általános Orvostudományi Kar Debrecen.

出版信息

Orv Hetil. 2017 Jan;158(3):111-116. doi: 10.1556/650.2017.30638.

Abstract

INTRODUCTION

In order to establish and use a national registry, several Hungarian hematology centers collected data of myeloproliferative neoplasia patients.

AIM

The recent publication is an analysis of the data of registered essential thrombocythaemic patients.

METHOD

an online electronic registry has been established, using 2008 World Health Organization's diagnostic criteria and thrombotic risk was evaluated according to Landolfi stratification.

RESULTS

Data of 350 essential thrombocythaemic patients from 15 Hungarian hematology centers entered up to the date of June 30, 2015 were used for analysis. Patients were followed up to (median) 6 years. The epidemiologic data (age, gender) and thrombotic events prior and after the diagnosis, were similar to the literature. The thrombotic events of anagrelide treated patient (n = 139) and the hydroxyurea + aspirin treated patients (n = 141) have been compared. The major arterial and venous events were similar between the groups, but there were fivefold less minor arterial and venous events in the anagrelide group (p<0.001). Thrombotic incidence after diagnosis were influenced only by medication and thrombotic events before the diagnosis.

CONCLUSIONS

Anagrelide significantly decreased the number of patients experiencing minor arterial and minor venous thrombosis, vs hydroxyurea + aspirin. Despite of the treatment the risk of thrombotic events after diagnosis remained high, and was significantly increased in patients with thrombosis before diagnosis. Orv. Hetil., 2017, 158(3), 111-116.

摘要

引言

为了建立和使用国家登记系统,匈牙利的几个血液学中心收集了骨髓增殖性肿瘤患者的数据。

目的

最近的出版物是对登记的原发性血小板增多症患者数据的分析。

方法

建立了一个在线电子登记系统,采用2008年世界卫生组织的诊断标准,并根据兰多尔菲分层法评估血栓形成风险。

结果

截至2015年6月30日,来自匈牙利15个血液学中心的350例原发性血小板增多症患者的数据被用于分析。患者随访(中位数)6年。流行病学数据(年龄、性别)以及诊断前后的血栓形成事件与文献报道相似。比较了使用阿那格雷治疗的患者(n = 139)和使用羟基脲+阿司匹林治疗的患者(n = 141)的血栓形成事件。两组之间主要动脉和静脉事件相似,但阿那格雷组的轻微动脉和静脉事件减少了五倍(p<0.001)。诊断后的血栓形成发生率仅受药物治疗和诊断前血栓形成事件的影响。

结论

与羟基脲+阿司匹林相比,阿那格雷显著减少了发生轻微动脉和轻微静脉血栓形成的患者数量。尽管进行了治疗,但诊断后血栓形成事件的风险仍然很高,并且在诊断前有血栓形成的患者中显著增加。《匈牙利医学周报》,2017年,158(3),111 - 116。

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