Regional Reference Center for Inherited Bleeding Disorders, University Hospital of Parma, Parma, Italy.
J Thromb Haemost. 2012 Jan;10(1):90-5. doi: 10.1111/j.1538-7836.2011.04566.x.
The increased life expectancy of the hemophilia population, primarily as a result of advances in factor replacement therapy, has enabled hemophiliacs to reach an older age. Consequently, age-related diseases, such as cardiovascular disorders and cancers, are being increasingly recognized in such patients. However, only few data are available on such co-morbidities, their management and impact on the primary bleeding disorders.
With the aim of investigating several still unclear issues regarding cancers in hemophilia patients, we conducted, on behalf the Italian Association of Hemophilia Centers (AICE), a study on cancers among Italian hemophiliacs.
Data pertaining to 122 hemophiliacs with 127 cancers between 1980 and 2010 were retrospectively collected in 21 centers of the AICE which chose to participate.
Sixty-nine percent of cancers were recorded during the decade 2001–2010. Eighty-three percent of patients were infected with hepatitis C virus (HCV) and 22% of them were also co-infected with human immunodeficiency virus (HIV). Forty-three percent of cancers were HCV-related, whereas 9%were HIV related. Virus-related cancers were more frequent and non-virus-related cancers less frequent in patients with severe hemophilia than in those with mild/moderate forms (P = 0.0004). The non-virus-related standardized mortality ratio (SMR) was 0.3. Hemorrhagic complications occurred more frequently in patients undergoing chemotherapy (14%) or radiotherapy (19%).
The results of the present study confirm that cancers have become a new challenge for physicians working in hemophilia centers and underline the need for prospective trials to better assess the epidemiology and to optimize the management of hemophiliacs with cancer.
由于因子替代疗法的进步，血友病患者的预期寿命延长，这使得血友病患者能够达到更高的年龄。因此，与年龄相关的疾病，如心血管疾病和癌症，在这些患者中越来越被认识到。然而，关于这些合并症及其管理以及对原发性出血性疾病的影响，仅有少数数据可用。
为了研究血友病患者癌症方面的一些仍不清楚的问题，我们代表意大利血友病中心协会（AICE）进行了一项针对意大利血友病患者癌症的研究。
在 21 个选择参与的 AICE 中心中，回顾性地收集了 1980 年至 2010 年间 122 名血友病患者和 127 例癌症的数据。
69%的癌症发生在 2001-2010 年这十年间。83%的患者感染了丙型肝炎病毒（HCV），其中 22%的患者还同时感染了人类免疫缺陷病毒（HIV）。43%的癌症与 HCV 相关，而 9%与 HIV 相关。与病毒相关的癌症在重型血友病患者中比在轻度/中度血友病患者中更为常见，而非病毒相关的癌症则较少（P=0.0004）。非病毒相关癌症的标准化死亡率比（SMR）为 0.3。在接受化疗（14%）或放疗（19%）的患者中，出血并发症更为常见。
本研究的结果证实，癌症已成为血友病中心医生面临的新挑战，并强调需要前瞻性试验来更好地评估其流行病学，并优化癌症血友病患者的管理。
