Van Creveldkliniek, Department of Hematology, University Medical Center Utrecht, Utrecht, The Netherlands.
Thromb Res. 2012 Aug;130(2):157-62. doi: 10.1016/j.thromres.2011.12.012. Epub 2011 Dec 30.
With increasing life expectancy, more haemophilia patients will be confronted with age-related problems. To ensure optimal care, it is important to know the occurrence of both fatal and non-fatal cardiovascular disease, malignancies and other types of co-morbidity in these patients. Our aim was to retrospectively assess the occurrence of co-morbidity and causes of death in a substantial birth-cohort of haemophilia patients.
Data on all types of co-morbidity were collected from medical records of 408 haemophilia patients (204 severe, 204 non-severe) born before 1971, and compared with the Dutch age-matched general male population.
Ten patients had 11 myocardial infarctions, none of which were fatal. The cumulative incidence of non-fatal myocardial infarction was significantly lower in patients with severe haemophilia than in the general population (0.5% versus 4.8%), but was not decreased in patients with non-severe haemophilia (4.4%). Intracranial bleeding occurred significantly more often in haemophilia patients. The occurrence of non-virus related malignancies, and other non-virus related co-morbidities was similar in haemophilia patients and the general population. HIV infection was present in 12% of patients, and hepatitis C infection in 56%. Seventy-eight patients (19%) were deceased. Main causes of death were malignancies, AIDS, hepatitis C, and intracranial bleeding.
Our results showed a decreased occurrence of myocardial infarction in patients with severe haemophilia, suggesting a protective effect of very low clotting factor levels on thrombotic cardiac events. No differences were found between haemophilia patients and the general population in the occurrence of any other type of non-virus related co-morbidity.
随着预期寿命的延长,越来越多的血友病患者将面临与年龄相关的问题。为了确保提供最佳的护理,了解这些患者致命和非致命心血管疾病、恶性肿瘤和其他类型合并症的发生情况非常重要。我们的目的是回顾性评估大量血友病患者发病队列中合并症的发生情况和死亡原因。
从出生于 1971 年以前的 408 名血友病患者(204 名严重,204 名非严重)的病历中收集所有类型合并症的数据,并与荷兰年龄匹配的普通男性人群进行比较。
10 名患者发生了 11 例心肌梗死,无致命性病例。严重血友病患者非致命性心肌梗死的累积发生率明显低于普通人群(0.5%对 4.8%),但非严重血友病患者的发生率并未降低(4.4%)。颅内出血在血友病患者中更为常见。非病毒相关恶性肿瘤和其他非病毒相关合并症的发生在血友病患者和普通人群中相似。12%的患者感染了 HIV,56%的患者感染了丙型肝炎。78 名患者(19%)死亡。死亡的主要原因是恶性肿瘤、艾滋病、丙型肝炎和颅内出血。
我们的结果表明,严重血友病患者心肌梗死的发生减少,提示极低凝血因子水平对血栓性心脏事件具有保护作用。血友病患者与普通人群在任何其他类型非病毒相关合并症的发生方面没有差异。