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骨髓增生异常综合征(MDS)是一组密切相关的异源性造血组织克隆疾病,其特征是骨髓细胞的成熟障碍伴无效血细胞生成。目前仍无满意的治疗方法。主要依靠输血以治疗贫血和血小板减少及使用抗生素治疗感染。13-顺式维甲酸(13-CRA)对MDS 有一定疗效,但需要长期治疗。而只有1/3的病人能耐受6个月的疗程。本文研究的目的在于观察长期使用13-CRA 对 MDS 的疗效及耐受性;并加用维生素 E,以观察是否能减轻 13-CRA 的毒性。病人和方法66例按 FAB 分型诊断 MDS 成立。其中原发性61例,其它肿瘤化疗后继发的MDS 5例。病程中位时间13.4(0.25~120)个月,中位年龄66(29~88)岁,男43、女23例。66例均接受13-CRA治疗,剂量100mg/M~2/d。①组21例单用13-CRA 治疗;②组45例,用13-CRA 加维生素 E 800mg/d 治疗。治疗6个月后比较两组病人的治疗反应、毒性、白血病转化率及生存期。治疗不满
Myelodysplastic syndrome (MDS) is a closely related group of hereditary hematopoietic clonal diseases characterized by maturation of myeloid cells with inefficient hematopoiesis. There is still no satisfactory treatment. Mainly rely on blood transfusion to treat anemia and thrombocytopenia and the use of antibiotics to treat the infection. 13-cis retinoic acid (13-CRA) has some effect on MDS, but requires long-term treatment. Only one-third of patients tolerate 6 months of treatment. The purpose of this study is to observe the long-term use of 13-CRA on the efficacy and tolerability of MDS; and vitamin E, to observe whether it can reduce the toxicity of 13-CRA. Sixty-six patients were diagnosed according to FAB MDS. Including 61 cases of primary, other tumors after chemotherapy in 5 cases of MDS. Duration of the median time of 13.4 (0.25 ~ 120) months, the median age of 66 (29 to 88) years old, 43 males and 23 females. 66 cases received 13-CRA treatment, the dose of 100mg / M ~ 2 / d. ① 21 cases were treated with 13-CRA alone; ② 45 cases were treated with 13-CRA plus 800 mg / day of vitamin E; The treatment response, toxicity, leukemia conversion rate, and survival were compared between the two groups after 6 months of treatment. Treatment dissatisfaction