摘要
目的回顾性分析550例初诊骨髓增生异常综合征（MDS）患者的临床特点、细胞遗传学特征和生存情况。方法收集患者基本信息，采用WHO（2008）标准进行分型，同时应用国际预后积分系统（IPSS）、基于WHO造血组织肿瘤分类的预后积分系统（WPSS）及修订版IPSS（IPSS-R）积分系统进行预后分层。结果550例MDS患者中位年龄57（12-89）岁，男女比为1.72∶1。血液学特点：外周血细胞三系可有不同程度减少或增加，中位HGB为72（22-154）g/L，中位PLT为52（3-587）×10^9/L，中位WBC为2.52（0.11-48.00）×10^9/L。细胞遗传学特征：251例（45.6%）检出克隆性染色体异常，复杂核型89例，占所有患者的16.2%。染色体异常类型主要以不平衡异常为主，三体或单体多见。最常见的克隆性异常是＋8，占染色体异常患者的29.9%（75/251）；其次为-7/del（7q）、del（5q）、del（20q）等。550例患者中位随访27（0.3-144）个月，采用IPSS、IPSS-R、WPSS分别对患者进行分组并比较各组生存，差异均有统计学意义（P〈0.001）。其中IPSS-R分组中低危组中位OS期未达到，中危组中位OS期为44（95% CI 28-60）个月，高危组中位OS期为17（95% CI 13-21）个月，极高危组中位OS期为8（95% CI 5- 11）个月（P 〈0.001）。结论该研究中纳入的MDS患者中位年龄57（12-89）岁，细胞遗传学异常类型与欧美报道有所差异，IPSS、IPSS-R和WPSS分层对于预测MDS患者的生存有重要意义。
ObjectiveTo retrospectively analyze the clinical features, cytogenetic characteristics and survival of 550 newly diagnosed myelodysplastic syndrome（MDS）patients.MethodBy using WHO（2008）criterion for classification, the prognosis of stratification of 550 patients were evaluated according to the IPSS/WPSS/IPSS-R.ResultsThe median age was 57 years old（range： 12-89 years old）. The ratio of male and female was 1.72. In all patients, the median hemoglobin level was 72（22-154）g/L, the median platelet count was 52（3-587）×10^9/L and the median WBC was 2.52（0.11-48.00）×10^9/L. 45.6%（251/550）patients had clonal chromosome abnormalities, in which 89（16.2%）patients with complex karyotype. The types of chromosome aberration were mainly dominated by unbalanced anomalies, and the most common types of chromosome aberration were trisomy or monosomy. The most common abnormity was ＋8. Other aberrations in frequent order were-7/del（7q）, del（5q）, del（20q）, and so on. In addition, 550 patients were evaluated according to IPSS, IPSS-R, and WPSS prognostic scoring system. The results showed that the IPSS, WPSS and IPSS-R scores were significantly affected OS（P 〈0.001）. The median OS in the IPSSR Low, Intermediate, High and Very Highrisk groups was not reached, 44（95%CI 28-60）, 17（95%CI 13-21）and 8（95%CI 5-11）months, respectively.ConclusionIn this study, the patients＇ median age was 57（12-89）years old, and the cytogenetic characteristics were significantly different from western MDS patients. IPSS, IPSS-R and WPSS were important in predicting the survival of MDS patients.
出处
《中华血液学杂志》
CAS
CSCD
北大核心
2016年第10期864-869,共6页
Chinese Journal of Hematology
基金
国家自然科学基金（81570138）
江苏省科技支撑计划重点项目（BE2015639）
