Maung Su W, Burke Cathie, Hayde Jennifer, Walshe Janice, McDermott Ray, Desmond Ronan, McHugh Johnny, Enright Helen
a Department of Haematology , Tallaght Hospital , Dublin , Ireland.
b Department of Pharmacy , Tallaght Hospital , Dublin , Ireland.
Hematology. 2017 Jul;22(6):341-346. doi: 10.1080/10245332.2017.1286539. Epub 2017 Feb 15.
To demonstrate the incidence, characteristics, treatment and outcomes of patients with therapy-related myelodysplastic syndromes and therapy-related acute myeloid leukaemia (t-MDS/AML) in a tertiary referral centre.
Patients meeting the diagnostic criteria for t-MDS/AML from 2003 to 2014 were reviewed to analyse their diagnostic features, details of antecedent disorder and treatment, approach to management and survival.
39 patients who developed t-MDS/AML were identified with incidence of 8.7%. Median age and gender distribution were similar to de novo MDS but t-MDS/AML patients had greater degree of cytopenia and adverse karyotypes. Time to development of t-MDS/AML was shortest for patients with antecedent haematological malignancy compared to solid tumours and autoimmune disorders (46, 85 and 109 months). Patients with prior acute leukaemia had the shortest latency and poor overall survival. Treatment options included best supportive care (56%), Azacitidine (31%) or intensive chemotherapy/allogeneic transplant (13%). Median OS of all patients was 14 months. Survival declined markedly after two years and 5-year OS was 13.8%. Longer survival was associated with blast count <5% at diagnosis, previous haematological disorder, lower risk IPSS-R and a normal karyotype. Four out of five patients who received intensive therapy/transplant remain alive with median OS of 14 months. Median OS of Azacitidine-treated group was 11 months.
t-MDS/AML patients showed unique characteristics which influenced their treatment and outcomes. IPSS-R may be useful in risk-adapted treatment approaches and can predict outcomes. Survival remains poor but improved outcomes were seen with allogeneic transplantation. Azacitidine may be effective in patients unfit for intensive therapies.
在一家三级转诊中心展示治疗相关的骨髓增生异常综合征和治疗相关的急性髓系白血病（t-MDS/AML）患者的发病率、特征、治疗及转归。
对2003年至2014年符合t-MDS/AML诊断标准的患者进行回顾，分析其诊断特征、既往疾病详情及治疗情况、管理方法和生存情况。
共识别出39例发生t-MDS/AML的患者，发病率为8.7%。中位年龄和性别分布与原发性MDS相似，但t-MDS/AML患者的血细胞减少程度更严重，核型异常更常见。与实体瘤和自身免疫性疾病患者相比，既往有血液系统恶性肿瘤的患者发生t-MDS/AML的时间最短（分别为46、85和109个月）。既往有急性白血病的患者潜伏期最短，总生存期较差。治疗选择包括最佳支持治疗（56%）、阿扎胞苷（31%）或强化化疗/异基因移植（13%）。所有患者的中位总生存期为14个月。两年后生存率显著下降，5年总生存率为13.8%。较长的生存期与诊断时原始细胞计数<5%、既往血液系统疾病、较低的国际预后评分系统修订版（IPSS-R）风险和正常核型相关。接受强化治疗/移植的五分之四患者仍存活，中位总生存期为14个月。阿扎胞苷治疗组的中位总生存期为11个月。
t-MDS/AML患者表现出独特的特征，这些特征影响了他们的治疗和转归。IPSS-R可能有助于风险适应性治疗方法，并可预测转归。生存率仍然较低，但异基因移植的转归有所改善。阿扎胞苷可能对不适合强化治疗的患者有效。
