Groupe Francophone des Myélodysplasies, Hôpital Avicenne, Bobigny, France.
Haematologica. 2010 Jun;95(6):892-9. doi: 10.3324/haematol.2009.014357. Epub 2009 Dec 16.
There is little published information on the everyday clinical management of myelodysplastic syndromes in real world practice.
We conducted a cross-sectional study of all patients with myelodysplastic syndromes attending 74 French centers in a 1-week period for inpatient admission, day-hospital care or outpatient visits.
Nine hundred and seven patients were included; 67.3% had lower-risk myelodysplastic syndromes (International Prognostic Scoring System: low or intermediate-1). Karyotype had been analyzed in 82.5% of the cases and was more often of intermediate or poor risk in patients under 65 years old compared with those who were older. Red blood cell transfusions accounted for as many as 31.4% of the admissions. Endogenous erythropoietin level was less than 500 IU/L in 88% of the patients tested. Erythroid stimulating agents had been or were being used in 36.8% of the lower risk patients, iron chelation in 31% of lower risk patients requiring red blood cell transfusions and lenalidomide in 41% of lower risk patients with del 5q. High-dose chemotherapy, hypomethylating agents, low dose cytarabine and allogeneic stem cell transplantation had been or were being used in 14.8%, 31.1%, 8.8% and 5.1%, respectively, of higher-risk patients.
Karyotype is now assessed in most patients with myelodysplastic syndromes, and patients under 65 years old may have more aggressive disease. Apart from erythroid-stimulating agents and, in higher-risk myelodysplastic syndromes, hypomethylating agents, specific treatments are used in a minority of patients with myelodysplastic syndromes and red blood cell transfusions still represent the major reason for hospital admission.
关于骨髓增生异常综合征在真实世界实践中的日常临床管理,发表的信息很少。
我们对在一周内因住院、日间住院或门诊就诊而在 74 家法国中心就诊的所有骨髓增生异常综合征患者进行了横断面研究。
共纳入 907 例患者;67.3%为低危骨髓增生异常综合征(国际预后评分系统:低危或中危-1)。82.5%的病例分析了核型,年龄<65 岁的患者核型更倾向于中危或高危,而年龄较大的患者核型更倾向于低危。红细胞输血占入院人数的 31.4%。接受检测的患者中,88%的内源性促红细胞生成素水平<500IU/L。36.8%的低危患者使用了红细胞刺激剂,31%需要输血的低危患者使用了铁螯合剂,41%的低危 del5q 患者使用了来那度胺。14.8%、31.1%、8.8%和 5.1%的高危患者分别接受了大剂量化疗、低甲基化药物、低剂量阿糖胞苷和异基因干细胞移植。
现在对大多数骨髓增生异常综合征患者进行核型评估,年龄<65 岁的患者可能患有更具侵袭性的疾病。除了红细胞刺激剂和高危骨髓增生异常综合征中的低甲基化药物外,少数骨髓增生异常综合征患者使用了特定的治疗方法,红细胞输血仍然是住院的主要原因。