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真性红细胞增多症:与性别相关的表型差异。

Polycythemia vera: gender-related phenotypic differences.

机构信息

Institute of Internal Medicine and Geriatrics, Haemostasis Research Center, Catholic University School of Medicine, Rome, Italy.

出版信息

Intern Emerg Med. 2012 Dec;7(6):509-15. doi: 10.1007/s11739-011-0634-3. Epub 2011 Jun 10.

DOI:10.1007/s11739-011-0634-3
PMID:21660494
Abstract

In polycythemia vera, gender has recently been shown to influence the JAK2(V617F) allele burden, but its effect on the disease phenotype is unknown. This issue was investigated using the database of the European Collaboration on Low-dose Aspirin in Polycythemia Vera (ECLAP) Study. The ECLAP Study recruited 1,638 polycythemic subjects and followed for 2.7 ± 1.3 years. At study entry, men, compared to women, had a higher prevalence of myocardial infarction (11.3 vs. 5.8%; P < 0.0001) and peripheral arterial disease (6.1 vs. 2.9%; P < 0.05) while a history of venous thrombosis was more common in women (11.4 vs. 7.9%, P = 0.016). Among 234 venous thrombosis, there were 39 splanchnic vein thromboses (33 extra-hepatic portal vein thromboses and 6 Budd-Chiari syndromes). Most of these events occurred as an early disease presentation in young female subjects. Women, compared to men, had higher platelet counts (average value 430 ± 213 vs. 375 ± 201 × 10(9)/L; P < 0.0001) and lower hematocrits (0.46 ± 0.06 vs. 0.48 ± 0.06 l/l; P < 0.0001). Cholesterol plasma level, available in 995 subjects (61%), was lower in male patients (180.8 ± 43.1 vs. 196 ± 46.6 mg/dl; P < 0.0001). During follow-up there were 205 major thromboses confirming an high incidence of myocardial infarction in men although not statistically significant (1.2 vs. 0.6 cases per 100 person-years; P > 0.05). These data show several gender-related differences both in the thrombotic diathesis and in the prevalence of vascular risk factors of PV patients.

摘要

在真性红细胞增多症中,最近有研究表明性别会影响 JAK2(V617F)等位基因负担,但它对疾病表型的影响尚不清楚。本研究使用欧洲低剂量阿司匹林治疗真性红细胞增多症研究(ECLAP)数据库进行了此项研究。ECLAP 研究招募了 1638 例真性红细胞增多症患者,并随访了 2.7±1.3 年。在研究入组时,与女性相比,男性更易发生心肌梗死(11.3%比 5.8%;P<0.0001)和外周动脉疾病(6.1%比 2.9%;P<0.05),而女性更易发生静脉血栓形成病史(11.4%比 7.9%,P=0.016)。在 234 例静脉血栓形成中,有 39 例为内脏静脉血栓形成(33 例肝外门静脉血栓形成和 6 例布加综合征)。这些事件中的大多数发生在年轻女性患者的早期疾病表现中。与男性相比,女性的血小板计数更高(平均 430±213 比 375±201×109/L;P<0.0001),血细胞比容更低(0.46±0.06 比 0.48±0.06 l/l;P<0.0001)。234 例中有 995 例(61%)患者可提供胆固醇血浆水平,男性患者的胆固醇水平较低(180.8±43.1 比 196±46.6 mg/dl;P<0.0001)。随访期间有 205 例发生主要血栓事件,证实男性心肌梗死发生率较高,但无统计学意义(1.2 比 0.6 例/100 人年;P>0.05)。这些数据表明,真性红细胞增多症患者在血栓形成倾向和血管危险因素的流行方面存在多种与性别相关的差异。

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Splanchnic vein thrombosis: clinical presentation, risk factors and treatment.内脏静脉血栓形成:临床表现、危险因素和治疗。
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