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真性红细胞增多症的流行病学数据:一项842例病例的研究。

Epidemiological data in polycythaemia vera: a study of 842 cases.

作者信息

Najean Y, Rain J D, Billotey C

机构信息

Service de Médecine Nucléaire, Hôpital Saint-Louis, Paris, France.

出版信息

Hematol Cell Ther. 1998 Aug;40(4):159-65.

PMID:9766920
Abstract

An epidemiological study of 842 polycythaemic patients (entered between 1980 and 1997 in the French investigational prospective protocols) is presented. The global incidence is approximately 0.8-1.5/100,000/year in the reference area (Ile-de-France and surrounding areas). It increases linearly with age until 80, which suggests that several mutational somatic events are necessary. There was a slight male excess (sex-ratio 1.2, after correction for the percentage of male and female French people still living at risk). We did observe a slight excess of PV in the population of Jewish ancestry. A surprising excess of former blood donors (20.7% of the PV cases, compared to 8% estimated in the reference population) was observed. Only a few cases of familial myeloproliferative diseases and occurence of leukemia in the family of our patients have been observed; even if slight, this excess is statistically significant. In contrast, no excess of carcinomas was observed either in the family or in the patients' antecedents. We did not find any excess of radiation exposure in our cases. When analysing the previous occupation of our patients a possible excess of physicians and of patients previously working in occupations using solvents and glues was found, but this finding needs confirmation.

摘要

本文介绍了一项对842例红细胞增多症患者的流行病学研究(这些患者于1980年至1997年纳入法国前瞻性研究方案)。在参考区域(法兰西岛及周边地区),总体发病率约为每年0.8 - 1.5/100,000。发病率随年龄增长呈线性上升,直至80岁,这表明需要多个体细胞突变事件。男性略多(校正仍处于风险中的法国男性和女性百分比后,性别比为1.2)。我们确实观察到犹太血统人群中真性红细胞增多症略多。观察到既往献血者比例惊人地高(真性红细胞增多症病例的20.7%,而参考人群中的估计比例为8%)。在我们的患者家族中仅观察到少数家族性骨髓增殖性疾病病例和白血病发生;即使数量很少,这种增多在统计学上也具有显著性。相比之下,在家族或患者既往史中未观察到癌症增多。在我们的病例中未发现辐射暴露过多。分析患者既往职业时,发现医生以及既往从事使用溶剂和胶水职业的患者可能略多,但这一发现需要证实。

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