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：

骨髓增生異常綜合征; 乳酸脫氫酶; 鐵蛋白; 細胞遺傳學; 臨床分析

DOI：

10.7507/1002-0179.20130309

Video：

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目的　探討骨髓增生異常綜合征（MDS）患者的臨床特點。方法　選取我院2008年3月－2012年10月確診為MDS的231例患者臨床資料進行回顧性分析。患者年齡21～87歲，中位年齡59歲。結果　231例患者中，難治性血細胞減少伴多系發育異常（RCMD）最多見，占45.0%（104/231）；以貧血乏力癥狀就診多見占66.7%（154/231）；血常規中以全血細胞均減少多見占61%（141例/231例）；網織紅細胞以正常或增高為主占61%（141/231）；低熒光值增高多見62%（144/231）。乳酸脫氫酶和鐵蛋白在各診斷亞型及各國際預后積分系統（IPSS）評分間存在差異，其中乳酸脫氫酶在難治性貧血伴原始細胞增多2型（RAEB-2）中高于綜合組：難治性貧血（RA）、難治性貧血伴環狀鐵粒幼細胞（RAS）、5q?綜合征及RCMD相比較差異有統計學意義（P＜0.05），高危組乳酸脫氫酶高于中危1組及中危2組，其差異有統計學意義（P＜0.05），高危組鐵蛋白高于中危1組其差異有統計學意義（P＜0.05），其余差異無統計學意義（P＞0.05）。染色體異常率為39%，其中20例為復雜染色體核型，IPSS評分中危1最多見為52.4%（55/105）。結論　MDS臨床表現多樣，缺乏特異性，需綜合骨髓涂片、活檢、細胞遺傳學的結果提高診斷率。

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

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.	趙惠玲, 劉雁, 劉舒平. 骨髓增生異常綜合征78例分析[J]. 實用醫技雜志, 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.	中美聯合上海市白血病協作組. 骨髓增生異常綜合征435例WHO分型和細胞遺傳學分析[J]. 中華內科雜志, 2008, 47(6): 464-467.
9.	陳書長, 朱紅, 廬榮, 等. 低增生骨髓增生異常綜合征的由來和轉歸[J]. 中華內科雜志, 1999, 38(6): 9-11.
10.	邱鏡瀅, 賴悅云, 柴曄, 等. 306例骨髓增生異常綜合征染色體核型的研究[J]. 中國實驗血液學雜志, 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.	顧敏, 陳其文, 周永明, 等. MDS患者血漿SF, LDH, β2-MG水平臨床觀察[J]. 臨床和實驗醫學雜志. 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. 　趙惠玲, 劉雁, 劉舒平. 骨髓增生異常綜合征78例分析[J]. 實用醫技雜志, 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. 　中美聯合上海市白血病協作組. 骨髓增生異常綜合征435例WHO分型和細胞遺傳學分析[J]. 中華內科雜志, 2008, 47(6): 464-467.
9. 　陳書長, 朱紅, 廬榮, 等. 低增生骨髓增生異常綜合征的由來和轉歸[J]. 中華內科雜志, 1999, 38(6): 9-11.
10. 　邱鏡瀅, 賴悅云, 柴曄, 等. 306例骨髓增生異常綜合征染色體核型的研究[J]. 中國實驗血液學雜志, 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. 　顧敏, 陳其文, 周永明, 等. MDS患者血漿SF, LDH, β2-MG水平臨床觀察[J]. 臨床和實驗醫學雜志. 2011, 10(7): 503-504.

West China Medical Journal

Clinical Analysis of Myelodysplastic Syndrome

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