Clinical Analysis of Myelodysplastic Syndrome_West China Medical Journal

Authors：

XIONG Mei ,  ZHU Huanling , XIE Liping , WANG Yuchun , HE Chuan , JI Jie , LIAO Xiaomei , CHEN Xinchuan , LI Jianjun , CUI Xu , GONG Yuping , LIU Ting.

Department of Hematology, West China Hospital, Sichuan University, Chengdu, Sichuan 610041, P. R. China;

Corresponding author：

ZHU Huanling, Email: xiongmei67@yahoo.cn

Keywords：

骨髓增生异常综合征; 乳酸脱氢酶; 铁蛋白; 细胞遗传学; 临床分析

DOI：

10.7507/1002-0179.20130309

Video：

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目的　探讨骨髓增生异常综合征（MDS）患者的临床特点。方法　选取我院2008年3月－2012年10月确诊为MDS的231例患者临床资料进行回顾性分析。患者年龄21～87岁，中位年龄59岁。结果　231例患者中，难治性血细胞减少伴多系发育异常（RCMD）最多见，占45.0%（104/231）；以贫血乏力症状就诊多见占66.7%（154/231）；血常规中以全血细胞均减少多见占61%（141例/231例）；网织红细胞以正常或增高为主占61%（141/231）；低荧光值增高多见62%（144/231）。乳酸脱氢酶和铁蛋白在各诊断亚型及各国际预后积分系统（IPSS）评分间存在差异，其中乳酸脱氢酶在难治性贫血伴原始细胞增多2型（RAEB-2）中高于综合组：难治性贫血（RA）、难治性贫血伴环状铁粒幼细胞（RAS）、5q?综合征及RCMD相比较差异有统计学意义（P＜0.05），高危组乳酸脱氢酶高于中危1组及中危2组，其差异有统计学意义（P＜0.05），高危组铁蛋白高于中危1组其差异有统计学意义（P＜0.05），其余差异无统计学意义（P＞0.05）。染色体异常率为39%，其中20例为复杂染色体核型，IPSS评分中危1最多见为52.4%（55/105）。结论　MDS临床表现多样，缺乏特异性，需综合骨髓涂片、活检、细胞遗传学的结果提高诊断率。

Citation： XIONG Mei,ZHU Huanling,XIE Liping,WANG Yuchun,HE Chuan,JI Jie,LIAO Xiaomei,CHEN Xinchuan,LI Jianjun,CUI Xu,GONG Yuping,LIU Ting.. Clinical Analysis of Myelodysplastic Syndrome. West China Medical Journal, 2013, 28(7): 994-997. doi: 10.7507/1002-0179.20130309 Copy

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6.	赵惠玲, 刘雁, 刘舒平. 骨髓增生异常综合征78例分析[J]. 实用医技杂志, 2006, 13( 09): 1488.
7.	Ulrich G, Strupp C, Giagounidis A. Clinical features and prognosis of patients with myelodysplastic syndromes[J]. Cancer Treat Rev, 2007, 33(1): S15-S18.
8.	中美联合上海市白血病协作组. 骨髓增生异常综合征435例WHO分型和细胞遗传学分析[J]. 中华内科杂志, 2008, 47(6): 464-467.
9.	陈书长, 朱红, 庐荣, 等. 低增生骨髓增生异常综合征的由来和转归[J]. 中华内科杂志, 1999, 38(6): 9-11.
10.	邱镜滢, 赖悦云, 柴晔, 等. 306例骨髓增生异常综合征染色体核型的研究[J]. 中国实验血液学杂志, 2004, 12(4): 455-459.
11.	Chen B, Zhao WL, Jin J, et al. Clinical and cytogenetic features of 508 Chinese patients with myelodysplastic syndrome and comparison with those in Western countries[J]. Leukemia, 2005, 19(5): 767-775.
12.	Nishino HT, Chang CC. Myelodysplastic syndromes: clinicopathologic features, pathobiology, and molecular pathogenesis[J]. Arch Pathol Lab Med, 2005, 129(10): 1299-1310.
13.	Germing U, Hildebrandt B, Pfeilstöcker M, et al. Refinement of the international prognostic scoring system (IPSS) by including LDH as an additional prognostic variable to improve risk assessment in patients with primary myelodysplastic syndromes (MDS)[J]. Leukemia, 2005, 19(12): 2223-2231.
14.	Malcovati L, Porta MG, Pascutto C, et al. Prognostic factors and Life expectancy in myelodysplastic syndromes classified according to WHO criteria: a basis for clinical decision making[J]. J Clin Oncol, 2005, 23(30): 7594-7603.
15.	顾敏, 陈其文, 周永明, 等. MDS患者血浆SF, LDH, β2-MG水平临床观察[J]. 临床和实验医学杂志. 2011, 10(7): 503-504.

1. 　Aul C, Bowen DT, Pathogenesis YY. Etiology and epidemiology of myelodysplastic syndromes[J]. Haematologica, 1998, 83(1): 71-86.
2. 　Tefferi A, Myelodysplastic VJ. Syndromes[J]. N Engl J Med, 2009, 361(19): 1872-1885.
3. 　Vardiman JW, Thiele J, Arber DA, et al. The 2008 revision of the World Health Organization (WHO) classification of myeloid neoplasms and acute leukemia: rationale and important changes[J]. Blood, 2009, 114(5): 937-951.
4. 　Greenberg P, Cox C, Lebeau MM, et al. International scoring system for evaluating prognosis in myelodysplastic syndromes[J]. Blood, 1997, 89(6): 2079-2088.
5. 　Ulrich G, Judith N, Rainer H. The epidemiology of myelodysplastic syndromes[J]. Clinical Leukemia, 2008, 2(1): 34-38.
6. 　赵惠玲, 刘雁, 刘舒平. 骨髓增生异常综合征78例分析[J]. 实用医技杂志, 2006, 13( 09): 1488.
7. 　Ulrich G, Strupp C, Giagounidis A. Clinical features and prognosis of patients with myelodysplastic syndromes[J]. Cancer Treat Rev, 2007, 33(1): S15-S18.
8. 　中美联合上海市白血病协作组. 骨髓增生异常综合征435例WHO分型和细胞遗传学分析[J]. 中华内科杂志, 2008, 47(6): 464-467.
9. 　陈书长, 朱红, 庐荣, 等. 低增生骨髓增生异常综合征的由来和转归[J]. 中华内科杂志, 1999, 38(6): 9-11.
10. 　邱镜滢, 赖悦云, 柴晔, 等. 306例骨髓增生异常综合征染色体核型的研究[J]. 中国实验血液学杂志, 2004, 12(4): 455-459.
11. 　Chen B, Zhao WL, Jin J, et al. Clinical and cytogenetic features of 508 Chinese patients with myelodysplastic syndrome and comparison with those in Western countries[J]. Leukemia, 2005, 19(5): 767-775.
12. 　Nishino HT, Chang CC. Myelodysplastic syndromes: clinicopathologic features, pathobiology, and molecular pathogenesis[J]. Arch Pathol Lab Med, 2005, 129(10): 1299-1310.
13. 　Germing U, Hildebrandt B, Pfeilstöcker M, et al. Refinement of the international prognostic scoring system (IPSS) by including LDH as an additional prognostic variable to improve risk assessment in patients with primary myelodysplastic syndromes (MDS)[J]. Leukemia, 2005, 19(12): 2223-2231.
14. 　Malcovati L, Porta MG, Pascutto C, et al. Prognostic factors and Life expectancy in myelodysplastic syndromes classified according to WHO criteria: a basis for clinical decision making[J]. J Clin Oncol, 2005, 23(30): 7594-7603.
15. 　顾敏, 陈其文, 周永明, 等. MDS患者血浆SF, LDH, β2-MG水平临床观察[J]. 临床和实验医学杂志. 2011, 10(7): 503-504.

West China Medical Journal

Clinical Analysis of Myelodysplastic Syndrome

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