Ematologia Pediatrica e Trapianto di Midollo, Ospedale San Camillo, Roma, Italy.
Br J Haematol. 2010 Jun;149(6):890-5. doi: 10.1111/j.1365-2141.2010.08194.x. Epub 2010 Apr 29.
In order to assess the epidemiology of Hepatitis-Associated Aplasia (HAA) and compare treatment outcome of HAA with non-HAA patients, we evaluated 3916 aplastic anaemia patients reported to the European Registry between 1990 and 2007. Year, month, season of diagnosis, type and outcome of first-line therapy were analysed. Prevalence of HAA (n = 214) in Europe was 5%. Compared to non-HAA patients, HAA patients were younger (15 vs. 20 years, P < 0.001), with a male prevalence (68% vs. 58% P = 0.002), and were treated earlier after diagnosis (46 vs. 62 d; P < 0.001). No significant differences were found regarding the year or month of diagnosis. No geographic clusters could be identified. Actuarial survival at 10 years after first-line immunosuppression was 69%, and did not differ according to aetiology. The 10-year actuarial survival after transplantation was 70%, and was comparable in HAA and non-HAA patients, when stratified for age and donor type. In a multivariate Cox analysis, increasing age and delayed treatment were significant negative indicators for survival. In conclusion, the incidence of HAA was 5% and was evenly distributed over time and geographic areas in Europe. Treatment outcome and predictive variables, were comparable in patients with or without HAA.
为了评估肝炎相关性再生障碍性贫血（HAA）的流行病学，并比较 HAA 与非 HAA 患者的治疗结果，我们评估了 1990 年至 2007 年间向欧洲登记处报告的 3916 例再生障碍性贫血患者。分析了诊断的年份、月份、季节、一线治疗的类型和结果。欧洲 HAA（n=214）的患病率为 5%。与非 HAA 患者相比，HAA 患者年龄更小（15 岁 vs. 20 岁，P < 0.001），男性患病率更高（68% vs. 58%，P = 0.002），诊断后接受治疗的时间更早（46 天 vs. 62 天；P < 0.001）。诊断的年份或月份无显著差异。未发现地理聚集现象。一线免疫抑制治疗 10 年后的累积生存率为 69%，与病因无关。移植后 10 年的累积生存率为 70%，在按年龄和供体类型分层时，HAA 和非 HAA 患者的生存率相当。多变量 Cox 分析显示，年龄增加和治疗延迟是生存的显著负性指标。总之，HAA 的发病率为 5%，在欧洲的时间和地理区域内分布均匀。HAA 与非 HAA 患者的治疗结果和预测变量相当。
