中华血液学杂志
中華血液學雜誌
중화혈액학잡지
Chinese Journal of Hematology
2015年
3期
216-220
,共5页
马力%李星鑫%张静%邵英起%聂能%黄振东%葛美丽%郑以州%曲东霞
馬力%李星鑫%張靜%邵英起%聶能%黃振東%葛美麗%鄭以州%麯東霞
마력%리성흠%장정%소영기%섭능%황진동%갈미려%정이주%곡동하
贫血,再生障碍性%克隆进化%骨髓增生异常综合征%白血病,髓样,急性%临床特征
貧血,再生障礙性%剋隆進化%骨髓增生異常綜閤徵%白血病,髓樣,急性%臨床特徵
빈혈,재생장애성%극륭진화%골수증생이상종합정%백혈병,수양,급성%림상특정
Anemia,aplastic%Clonal evolution%Myelodysplastic syndrome%Leukemia,myeloid,acute%Clinical characteristics
目的 观察获得性再生障碍性贫血(AA)患者克隆性演变为骨髓增生异常综合征/急性髓系白血病(MDS/AML)的临床特征及转归.方法 回顾性分析1994年12月至2011年12月进展为MDS/AML的19例AA患者临床特征及转归,包括治疗方案、治疗前后染色体核型变化、治疗反应及生存情况.结果 中位随访49(15～97)个月,进展为MDS/AML 19例AA患者中极重型AA10例、重型AA 8例、非重型AA1例.G-CSF治疗中位时间为270(29～510)d,进展后其染色体异常核型以-7为主(19例中11例),AA进展为MDS/AML中位时间为33(11～88)个月.AA治疗后有效组进展为MDS/AML中位时间(54.2个月)显著长于无效者(25.7个月),差异有统计学意义(t=3.677,P＜0.01).结论 AA患者进展为MDS/AML常伴有染色体核型异常,预后较差.
目的 觀察穫得性再生障礙性貧血(AA)患者剋隆性縯變為骨髓增生異常綜閤徵/急性髓繫白血病(MDS/AML)的臨床特徵及轉歸.方法 迴顧性分析1994年12月至2011年12月進展為MDS/AML的19例AA患者臨床特徵及轉歸,包括治療方案、治療前後染色體覈型變化、治療反應及生存情況.結果 中位隨訪49(15～97)箇月,進展為MDS/AML 19例AA患者中極重型AA10例、重型AA 8例、非重型AA1例.G-CSF治療中位時間為270(29～510)d,進展後其染色體異常覈型以-7為主(19例中11例),AA進展為MDS/AML中位時間為33(11～88)箇月.AA治療後有效組進展為MDS/AML中位時間(54.2箇月)顯著長于無效者(25.7箇月),差異有統計學意義(t=3.677,P＜0.01).結論 AA患者進展為MDS/AML常伴有染色體覈型異常,預後較差.
목적 관찰획득성재생장애성빈혈(AA)환자극륭성연변위골수증생이상종합정/급성수계백혈병(MDS/AML)적림상특정급전귀.방법 회고성분석1994년12월지2011년12월진전위MDS/AML적19례AA환자림상특정급전귀,포괄치료방안、치료전후염색체핵형변화、치료반응급생존정황.결과 중위수방49(15～97)개월,진전위MDS/AML 19례AA환자중겁중형AA10례、중형AA 8례、비중형AA1례.G-CSF치료중위시간위270(29～510)d,진전후기염색체이상핵형이-7위주(19례중11례),AA진전위MDS/AML중위시간위33(11～88)개월.AA치료후유효조진전위MDS/AML중위시간(54.2개월)현저장우무효자(25.7개월),차이유통계학의의(t=3.677,P＜0.01).결론 AA환자진전위MDS/AML상반유염색체핵형이상,예후교차.
Objective To analyze the clinical features of clonal evolution of acquired aplastic anemia (AA) into myelodysplastic syndrome/acute myeloid leukemia (AML) and review of literatures.Methods AA developing MDS/AML patients between December 1994 and December 2011 enrolled into this study to analyze their clinical characteristics.Results During the median follow-up of 49 (15-97) months,19 patients evolved to MDS/AML,of whom 10,8 and 1 were from VSAA,SAA and NSAA subgroups,respectively.The median G-CSF therapy was 270 (29-510) days.There were monosomy 7 in 11 (57.9％) of 19 patients with AA evolved to MDS/AML.The median AA evolved to MDS/AML was 33 (11-88) months.The median MDS/AML transformation in responders (54.2 months) was significantly longer than of non-responders (25.7 months,P＜0.01).Conclusion AA patients could evolved into MDS/ AML concomitant with abnormal karotype and worse prognosis.
