中华血液学杂志
中華血液學雜誌
중화혈액학잡지
Chinese Journal of Hematology
2008年
11期
723-727
,共5页
许晓倩%王健民%吕书晴%陈莉%杨建民%章卫平%宋献民%许燕群%龚胜蓝%侯军%倪雄
許曉倩%王健民%呂書晴%陳莉%楊建民%章衛平%宋獻民%許燕群%龔勝藍%侯軍%倪雄
허효천%왕건민%려서청%진리%양건민%장위평%송헌민%허연군%공성람%후군%예웅
骨髓增生异常综合征%WHO分型%国际预后积分系统
骨髓增生異常綜閤徵%WHO分型%國際預後積分繫統
골수증생이상종합정%WHO분형%국제예후적분계통
Myelodysplastic syndrome%WHO classification%International Prognostic Scoring System
目的 探讨骨髓增生异常综合征(MDS)患者WHO分型、临床及血液学特征,以及MDS患者国际预后积分系统(IPSS)分组特点.方法 采用WHO新分型方案对80例MDS患者进行诊断及分型.对确诊的MDS患者的临床表现、血常规结果、骨髓象及转归进行总结性分析.结果 MDS患者中位发病年龄为47岁,低于国外学者的报道.染色体异常率为35.14%,检出的异常核型分布以+8最多,不同于欧、美洲国家和日本的大样本研究结果.74例患者经IPSS分组及追踪观察,11例转化为急性白血病.单变量分析提示年龄、染色体、原始细胞比例及外周血细胞减少系列数对患者有预后意义.IPSS低危组与中危-Ⅰ组生存率差异无统计学意义(P>0.05),其余各组间差异有统计学意义(P<0.05).WHO分型中难治性贫血(RA)、难治性血细胞减少伴多系增生异常(RCMD)与RA伴有原始细胞过多(RAEB)之间生存率差异有统计学意义(P值均<0.05),RAEB-Ⅰ与RAEB-Ⅱ之间生存率差异有统计学意义(P<0.05).结论 该组MDS患者的发病年龄、WHO亚型分布、染色体异常检出率及分布与欧、美洲国家和日本不同.WHO分型将RAEB分为RAEB-Ⅰ和RAEB-Ⅱ,对临床诊断、治疗和预后有益.IPSS同样适用于中国人,对选择合适的治疗方案及提示预后具有重要意义.
目的 探討骨髓增生異常綜閤徵(MDS)患者WHO分型、臨床及血液學特徵,以及MDS患者國際預後積分繫統(IPSS)分組特點.方法 採用WHO新分型方案對80例MDS患者進行診斷及分型.對確診的MDS患者的臨床錶現、血常規結果、骨髓象及轉歸進行總結性分析.結果 MDS患者中位髮病年齡為47歲,低于國外學者的報道.染色體異常率為35.14%,檢齣的異常覈型分佈以+8最多,不同于歐、美洲國傢和日本的大樣本研究結果.74例患者經IPSS分組及追蹤觀察,11例轉化為急性白血病.單變量分析提示年齡、染色體、原始細胞比例及外週血細胞減少繫列數對患者有預後意義.IPSS低危組與中危-Ⅰ組生存率差異無統計學意義(P>0.05),其餘各組間差異有統計學意義(P<0.05).WHO分型中難治性貧血(RA)、難治性血細胞減少伴多繫增生異常(RCMD)與RA伴有原始細胞過多(RAEB)之間生存率差異有統計學意義(P值均<0.05),RAEB-Ⅰ與RAEB-Ⅱ之間生存率差異有統計學意義(P<0.05).結論 該組MDS患者的髮病年齡、WHO亞型分佈、染色體異常檢齣率及分佈與歐、美洲國傢和日本不同.WHO分型將RAEB分為RAEB-Ⅰ和RAEB-Ⅱ,對臨床診斷、治療和預後有益.IPSS同樣適用于中國人,對選擇閤適的治療方案及提示預後具有重要意義.
목적 탐토골수증생이상종합정(MDS)환자WHO분형、림상급혈액학특정,이급MDS환자국제예후적분계통(IPSS)분조특점.방법 채용WHO신분형방안대80례MDS환자진행진단급분형.대학진적MDS환자적림상표현、혈상규결과、골수상급전귀진행총결성분석.결과 MDS환자중위발병년령위47세,저우국외학자적보도.염색체이상솔위35.14%,검출적이상핵형분포이+8최다,불동우구、미주국가화일본적대양본연구결과.74례환자경IPSS분조급추종관찰,11례전화위급성백혈병.단변량분석제시년령、염색체、원시세포비례급외주혈세포감소계렬수대환자유예후의의.IPSS저위조여중위-Ⅰ조생존솔차이무통계학의의(P>0.05),기여각조간차이유통계학의의(P<0.05).WHO분형중난치성빈혈(RA)、난치성혈세포감소반다계증생이상(RCMD)여RA반유원시세포과다(RAEB)지간생존솔차이유통계학의의(P치균<0.05),RAEB-Ⅰ여RAEB-Ⅱ지간생존솔차이유통계학의의(P<0.05).결론 해조MDS환자적발병년령、WHO아형분포、염색체이상검출솔급분포여구、미주국가화일본불동.WHO분형장RAEB분위RAEB-Ⅰ화RAEB-Ⅱ,대림상진단、치료화예후유익.IPSS동양괄용우중국인,대선택합괄적치료방안급제시예후구유중요의의.
Objective To investigate the WHO classification, clinical and hematological features and risk group of International Prognostic Scoring System (IPSS) in patients with myelodysplastie syndrome (MDS). Methods The diagnosis and classification of MDS patients were defined according to the WHO classification. The clinical manifestations, hemogram, bone marrow biopsy and prognosis were retrospectively analyzed. Results The median age at diagnosis of MDS was 47 yrs being younger than that in some foreign reports. The frequency of abnormal karyotype was 35.14 % and + 8 was the must frequent abnormal karyo-type in our study. Eleven of 74 patients transformed into leukemia. Univariate analysis showed that age, chro-mosome abnormality, percentage of bone marrow blast cells and number of cytopenias were significantly relat-ed to prognosis. There was a statistical difference in cure survival rate between 1PSS subcategories (P < 0.05) except that between low- and intermediate Ⅰ - risk subcategory (P > 0.05). There were statistical differences for refractory anemia(RA) vs RA with excess blast(RAEB), refractory cytopenias with maltilineage dysplasia (RCMD) vs RAEB and RAEB-Ⅰ vs RAEB-Ⅱ (P<0.05). Conclusions There were differences in age of disease onset, distribution of WHO, sub-classification and abnormal karyotype in this cohort of MDS patients as compared with those in Europe and Japan. It is helpful in diagnosis, treatment and prognosis to divide RAEB into RAEB-Ⅰ and RAEB-Ⅱ. IPSS was well applicable in Chinese MDS patients.
