中华血液学杂志
中華血液學雜誌
중화혈액학잡지
Chinese Journal of Hematology
2013年
2期
98-103
,共6页
刘艳荣%常艳%阮国瑞%秦亚溱%赖悦云%石红霞%王亚哲%李玲娣%江滨%李金兰
劉豔榮%常豔%阮國瑞%秦亞溱%賴悅雲%石紅霞%王亞哲%李玲娣%江濱%李金蘭
류염영%상염%원국서%진아진%뢰열운%석홍하%왕아철%리령제%강빈%리금란
基因,NPM1%白血病,非淋巴细胞,急性%免疫表型分型%临床特征
基因,NPM1%白血病,非淋巴細胞,急性%免疫錶型分型%臨床特徵
기인,NPM1%백혈병,비림파세포,급성%면역표형분형%림상특정
Gene,NPM1%Leukemia,non-lymphoblastic,acute%Immunophenotype%Clinical characteristic
目的 在形态学亚型比例相似的情况下,比较NPM1基因突变阳性的急性髓系白血病AML(NPM1m+AML) 与NPM1基因突变阴性AML(NPM1m-AML)非特指型(NOS)间的免疫表型与临床特征.方法 利用多参数流式细胞术进行免疫分型检测;定量PCR方法检测NPM1基因 A、B、D型突变及多种白血病相关基因.NPM1m+AML 并进行免疫分型患者104例,NPM1m-AML NOS患者 97例.结果 NPM1m+AML与NPM1m-AML NOS患者间在女性患者比例、WBC、PLT、骨髓原始细胞比例、正常核型患者比例、WT1基因表达水平、FLT3-ITD突变阳性率和第1疗程诱导缓解率上差异均有统计学意义(P<0.05).免疫表型方面主要为早期分化标志CD34、HLA-DR、CD117、CD38,淋系标志CD4、CD7、CD19、CD2和髓细胞标志CD13、CD14、CD15的低表达,及CD123、CD33的高表达.其中只有CD34、HLA-DR、CD7和CD4的阳性率在NPM1m+AML患者明显低于NPM1m-AML NOS患者(P<0.05),其余均为阳性细胞数的明显不同(P<0.05).进一步对NPM1m+AML中M1/M2和M4/M5患者进行分析,结果M1/M2患者保留了女性为主及WT1基因表达水平较高的特点(P<0.05),免疫表型共有10个抗原的阳性细胞数明显不同(P<0.05),主要为在M4/M5中包括单核细胞标志及淋系标志高表达及CD117的低表达.其中HLA-DR、CD64、CD11b、CD10、CD15和CD4 6个抗原的阳性率在NPM1m+AML M4/M5中也明显高于M1/M2患者(P<0.05).结论 在形态学亚型构成比例相似的情况下,NPM1m+AML与NPM1m-AML NOS患者相比,其主要临床特征与文献报道的一致.但免疫表型存在明显不同,主要表现为早期祖细胞标志、髓细胞标志和淋系标志的差异.在NPM1m+AML中单核细胞相关抗原在M4/M5患者中高表达.
目的 在形態學亞型比例相似的情況下,比較NPM1基因突變暘性的急性髓繫白血病AML(NPM1m+AML) 與NPM1基因突變陰性AML(NPM1m-AML)非特指型(NOS)間的免疫錶型與臨床特徵.方法 利用多參數流式細胞術進行免疫分型檢測;定量PCR方法檢測NPM1基因 A、B、D型突變及多種白血病相關基因.NPM1m+AML 併進行免疫分型患者104例,NPM1m-AML NOS患者 97例.結果 NPM1m+AML與NPM1m-AML NOS患者間在女性患者比例、WBC、PLT、骨髓原始細胞比例、正常覈型患者比例、WT1基因錶達水平、FLT3-ITD突變暘性率和第1療程誘導緩解率上差異均有統計學意義(P<0.05).免疫錶型方麵主要為早期分化標誌CD34、HLA-DR、CD117、CD38,淋繫標誌CD4、CD7、CD19、CD2和髓細胞標誌CD13、CD14、CD15的低錶達,及CD123、CD33的高錶達.其中隻有CD34、HLA-DR、CD7和CD4的暘性率在NPM1m+AML患者明顯低于NPM1m-AML NOS患者(P<0.05),其餘均為暘性細胞數的明顯不同(P<0.05).進一步對NPM1m+AML中M1/M2和M4/M5患者進行分析,結果M1/M2患者保留瞭女性為主及WT1基因錶達水平較高的特點(P<0.05),免疫錶型共有10箇抗原的暘性細胞數明顯不同(P<0.05),主要為在M4/M5中包括單覈細胞標誌及淋繫標誌高錶達及CD117的低錶達.其中HLA-DR、CD64、CD11b、CD10、CD15和CD4 6箇抗原的暘性率在NPM1m+AML M4/M5中也明顯高于M1/M2患者(P<0.05).結論 在形態學亞型構成比例相似的情況下,NPM1m+AML與NPM1m-AML NOS患者相比,其主要臨床特徵與文獻報道的一緻.但免疫錶型存在明顯不同,主要錶現為早期祖細胞標誌、髓細胞標誌和淋繫標誌的差異.在NPM1m+AML中單覈細胞相關抗原在M4/M5患者中高錶達.
목적 재형태학아형비례상사적정황하,비교NPM1기인돌변양성적급성수계백혈병AML(NPM1m+AML) 여NPM1기인돌변음성AML(NPM1m-AML)비특지형(NOS)간적면역표형여림상특정.방법 이용다삼수류식세포술진행면역분형검측;정량PCR방법검측NPM1기인 A、B、D형돌변급다충백혈병상관기인.NPM1m+AML 병진행면역분형환자104례,NPM1m-AML NOS환자 97례.결과 NPM1m+AML여NPM1m-AML NOS환자간재녀성환자비례、WBC、PLT、골수원시세포비례、정상핵형환자비례、WT1기인표체수평、FLT3-ITD돌변양성솔화제1료정유도완해솔상차이균유통계학의의(P<0.05).면역표형방면주요위조기분화표지CD34、HLA-DR、CD117、CD38,림계표지CD4、CD7、CD19、CD2화수세포표지CD13、CD14、CD15적저표체,급CD123、CD33적고표체.기중지유CD34、HLA-DR、CD7화CD4적양성솔재NPM1m+AML환자명현저우NPM1m-AML NOS환자(P<0.05),기여균위양성세포수적명현불동(P<0.05).진일보대NPM1m+AML중M1/M2화M4/M5환자진행분석,결과M1/M2환자보류료녀성위주급WT1기인표체수평교고적특점(P<0.05),면역표형공유10개항원적양성세포수명현불동(P<0.05),주요위재M4/M5중포괄단핵세포표지급림계표지고표체급CD117적저표체.기중HLA-DR、CD64、CD11b、CD10、CD15화CD4 6개항원적양성솔재NPM1m+AML M4/M5중야명현고우M1/M2환자(P<0.05).결론 재형태학아형구성비례상사적정황하,NPM1m+AML여NPM1m-AML NOS환자상비,기주요림상특정여문헌보도적일치.단면역표형존재명현불동,주요표현위조기조세포표지、수세포표지화림계표지적차이.재NPM1m+AML중단핵세포상관항원재M4/M5환자중고표체.
Objective To compare the immunophenotypic and clinical characteristics between NPM1 mutated acute myeloid leukemia(AML) (NPM1m+AML) and unmutated AML(NPM1m-AML) not otherwise characterized (NOS) under similar FAB subtypes constituent ratio. Methods Immunophenotyping and NPM1 gene mutation type-A,B and D and other leukemic related fusion genes were detected by multiparamter flow cytometry and real time RT-PCR or PCR,respectively. 104 AML patients with NPM1m+AML and performed immunophenotyping assay were included, 97 with NPM1m-AML. Results There were significant difference between the two groups at presentation in terms of sex, white blood count(WBC), platelet counts(PLT),blast ratio,normal karyotype ratio,WT1 expression level,FLT3-ITD mutation positive rate and remission rate of first course of induction therapy(P<0.05). On the immunophenotype, the expression of early differentiation antigens (CD34, HLA-DR, CD117, CD38), lymphocytic antigens(CD7, CD4, CD19, CD2), myeloid and monocytic differentiation-associated antigens (CD13, CD14, CD15) were lower, and that of CD33 as well as CD123 were higher in NPM1m+AML patients. Among them, only CD34, HLA-DR, CD7, and CD4 positive cases were significantly lower in NPM1m+AML group than in NPM1m-AML group(P<0.05), the rest of them had significant difference in the number of positive cells(P<0.05). Above features were further analyzed between the M1/M2 and M4/M5 subgroups. M1/M2 cases retained the women prominent and had a higher WT1 expression level(P<0.05). The expression of monocytic differentiation-associated antigens including HLA-DR and lymphocytic antigens were higher and that of CD117 were lower in M4/M5 subtype(P<0.05). Among them, the positive rates of HLA-DR, CD64, CD11b, CD10, CD15, and CD4 were significantly higher in M4/M5 than in M1/M2 in NPM1m+AML group(P<0.05). Conclusion The most clinical characteristics in NPM1m+AML patients are consistent with reports, but some immunophenotype are different to the previous reports under similar FAB subtypes constituent ratio. The major immunophenotypic features of NPM1m+AML patients are lower expression f progenitor,myeloid and lymphoid lineage antigens. Monocytic differentiation-associated antigens are only higher expression in M4/M5 cases when comparison with M1/M2 cases within NPM1m+AML group.