骨髓增生异常综合征患者50例临床分析  

Myelodysplastic syndromes:a clinical analysis of 50 cases

作　　者：孟秀琴[1] 艾工文[1] 施菊妹[1] 

机构地区：[1]同济大学附属第十人民医院血液科,上海200072

出　　处：《上海医学》2010年第9期837-840,共4页Shanghai Medical Journal

基　　金：国家自然科学基金资助项目(30973450)

摘　　要：目的探讨骨髓增生异常综合征（MDS）的临床特征及其治疗与转归的关系。方法对50例MDS患者的临床表现、体征、血常规、骨髓细胞学、治疗、生存时间等进行回顾性分析。结果 7例难治性血细胞减少伴多系增生异常（MDS-RCMD）患者伴有自身免疫性疾病（其中成人Still’s病2例、干燥综合征2例、系统性红斑狼疮1例、类风湿关节炎2例）,给予雄激素、1α-羟基维生素D3、复方皂矾丸加泼尼松15~60mg/d治疗后,患者均获得血液学改善。18例患者伴有骨髓纤维化,伴有中重度骨髓纤维化的MDS患者的骨髓活组织病理检查显示造血组织明显活跃至极度活跃,巨核细胞病态造血更明显。其中8例MDS患者转为急性髓系白血病。伴有骨髓纤维化的难治性贫血（MDS-RA）和MDS-RCMD患者的平均生存期为（16±4）个月,显著短于不伴骨髓纤维化的MDS-RA和MDS-RCMD的（58±6）个月（P〈0.05）。12例MDS患者转为急性白血病,平均转化时间为（16±3）个月;其中MDS-RA转为急性白血病的时间为（36±5）个月,显著长于MDS-RCMD的（7±3）个月和难治性贫血伴原始细胞增多（MDS-RAEB）的（5±1）个月（P值均〈0.05）。转化前12例MDS患者中8例合并中度骨髓纤维化（Gomori＋＋）,其中4例使用免疫抑制剂（2例使用环孢素A,2例使用糖皮质激素）治疗;转化后采用高三尖杉酯碱＋阿糖胞苷（HA）或蒽环类药物＋阿糖胞苷（DA）或阿克拉霉素＋阿糖胞苷＋粒细胞集落刺激因子（CAG）方案治疗,1例获得部分缓解,1例获得完全缓解,3例放弃治疗,7例治疗无效死亡（5例死于肺部感染,2例死于颅内出血）。结论合并自身免疫性疾病的MDS患者在治疗中加用糖皮质激素后血液学得以改善。伴有中重度骨髓纤维化的MDS患者巨核细胞病态造血更明显,更易转化为急性髓系白血病或骨髓衰竭,生存期短,是MDS预后不良因素之一。Objective To investigate the relationship of clinical characteristics, treatment and the prognoses of patients with myelodysplastic syndromes（MDS）. Methods The clinical manifestation, signs, blood routine, cytology, treatment and prognoses of 50 patients with MDS were retrospectively analyzed. Results Seven patients with MDS-RCMD also had autoimmune diseases （two with adult still’s diseases, two with primary Sjogren’s syndrome, one with systemic lupus erythematosus, two with rheumatoid arthritis）. After treatment with Androgen , 1,25-（OH）2-vitamine D3, Chinese medicine （Fu Fang Zao Fan Wan） , and predisone （15-60 mg/d）, all the seven patients achieved hematological improvement. Eighteen patients （36%） also had bone marrow fibrosis. The bone marrow pathology in MDS with moderate and severe fibrosis showed that cellularity was significantly higher, and the dysmegakaryopoiesis was more pronounced. Eight patients with moderate BM fibrosis finally had acute leukemia. Life expectancy of MDS patients with fibrosis was （16±3） months, which was significantly shorter than that in those without fibrosis （58±6） months （P0.05）. Twelve MDS patients finally had acute myeloleukemia. Time to leukemic transformation was （36±5） months in MDS-RA group, compared with （7±3） months in MDS-RCMD and （5±1） months in MDS-RAEB groups （P0.05）. Before development acute leukemia, eight patients had moderate BM fibrosis （Gomori＋＋）, and five patients had used immunosuppressive drugs. One patient achieved partial remission, one achieved complete remission, and seven had no effect after chemotherapy. Three patients gave up. Five patients died of lung infection and two died of brain hemorrhage. Conclusions MDS patients accompanied by autoimmune diseases can achieve hematological improvement by prednisone and other therapies. MDS patients with moderate or severe bone marrow fibrosis have a more pronounced dysmegakaryopoiesis and shorter survival time, and they are liable to develop

关 键 词：骨髓增生异常综合征 临床特征 骨髓纤维化 

分 类 号：R55[医药卫生—血液循环系统疾病]