摘要
目的观察获得性再生障碍性贫血（AA）治疗后演变为骨髓增生异常综合征（MDS）和（或）急性髓系白血病（AML）的发生率并分析其危险因素。方法长期随访1991至2009年收治的1003例AA患者，观察疾病演变，并分析其进展为MDS／AML的可能危险因素，包括患者性别、年龄、病因、治疗前后染色体核型变迁、疾病严重程度、治疗方案及治疗反应等。结果1003例患者中位随访时间为62（2～423）个月，其5年总生存率为（78．0±1．0）％，共计27例转化为MDS／AML[非重型AA（NSAA）11例，重型AA（SAA）6例，超重型AA（VSAA）10例]。Kaplan-Meier估计法分析1003例AA患者10年MDS／AML转化率为（4．5±1．0）％，VSAA组10年MDS／AML转化率[（12．8±3．5）％]显著高于NSAA组[（4．1±1．9）％，P〈0．01]和SAA组[（3．5±1．4）％，P〈0．01]，而后二组问差异无统计学意义（P=0．616）。单因素及多因素分析均显示患者年龄〉40岁[RR=3．527（95％CI：1．598—7．784），P〈0．01]、VSAA[RR=5．122（95％CI：2．214～11．853），P〈0．01]、发病前射线、毒物、化学制剂等接触史[RR=3．401（95％CI：1．535—7．534），P〈0．01]及重组人粒细胞集落刺激因子（rhuG—CSF）疗程大于300d[RR=10．782（95％C1：4．600—25．269），P〈0．01]为AA转化为MDS／AML的危险因素。结论长期随访对于评估AA患者治疗后进展为MDS／AML至关重要，随访期间应制定规范监测策略，及时发现转化的MDS／AML并尽早采取相应措施阻断其进展。
Objective To assess the incidence and risk factors for evolution of acquired aplastic anemia （AA） into myelodysplastic syndrome/acute myeloid leukemia （MDS/AML）. Method A total of 1003 AA patients hospitalized in our institute hospital between January 1991 and December 2009 enrolled into this study. The incidence and risk factors for AA developing MDS/AML by the Kaplan-Meier method and Cox proportional hazards models, respectively. Results The median follow-up was 62 （2 - 423） months and the projected 5-year survival rate was （78.0 ± 1.0） %. Twenty-seven patients evolved to MDS/AML, of whom 11,6 and 10 were from NSAA, SAA and VSAA subgroups, respectively. The estimated cumulative incidence of MDS/AML transformation fur these 1003 patients after diagnosis was （4.5 ± 1.0）% at 10 year. The incidence of MDS/AML transformation in VSAA subgroup [ （ 12.8 ± 3.5 ） % ] was significantly higher than in NSAA subgroup [ （4.1 ± 1.9） % ] （P 〈 0.001 ） and SAA subgroup [ （3.5 ± 1.4 ） % ] （P = 0. 008 ）, but no difference between the latter two subgroups （P =0.616）. Age [RR =3. 527 （95% CI：I. 598 -7. 784） ,P 0. 002 ], severity of disease [ RR = 5. 122 （95% CI：2. 214 - 11. 853 ）, P 〈 0. 001 ], the duration （days） of rhuG-CSF therapy [RR = 10. 782 （95% CI：4. 600 -25. 269） ,P 〈0.001 ] and exposure to ray, chemicals or drugs [ RR = 3. 401 （95 % CI：I. 535 -7. 534）, P = 0. 003 ] were risk factors for the transformation in both univariate and multivariate analyses. Conclusion Long-term follow-up is essential to assess the incidence and risk factors for evolutions of acquired AA into MDS/AML, and to administer salvage therapy for transformation in time during follow-up.
出处
《中华血液学杂志》
CAS
CSCD
北大核心
2011年第7期463-467,共5页
Chinese Journal of Hematology
