Ph染色体阳性急性白血病细胞遗传学及临床研究  

Cytogenetic and Clinical Study of Philadelphia Chromosome Positive Adult Acute Leukemia

作　　者：邱镜滢[1] 朱伟 张艳[1] 陈珊珊[1] 江滨[1] 史惠琳[1] 师岩[1] 何琦[1] 党辉[1] 王德炳[1] 陆道培[1] 

机构地区：[1]北京大学人民医院.北京大学血液病研究所

出　　处：《中国实验血液学杂志》2005年第3期358-363,共6页Journal of Experimental Hematology

摘　　要：为分析79例成人Ph染色体阳性急性白血病(Philadelphiachromosomepositiveacuteleukemia,Ph+AL)的细胞遗传学和相关临床表现及预后,联合应用细胞形态学、免疫分型,骨髓细胞染色体G显带技术(morphology,immunology,cytogenetics,MIC),对1991年10月-2003年12月住本院的79例Ph染色体阳性急性白血病进行了随访。结果表明:Ph+AL总的检出率为6.9%,其中Ph染色体阳性急性淋巴细胞性白血病(Philadelphiachromosomepositiveacutelymphoblasticleukemia,Ph+ALL)56例,检出率18%,Ph染色体阳性急性髓细胞性白血病(Philadelphiachromosomepositiveacutemyeloidleukemia,Ph+AML)10例,检出率1.2%。Ph染色体阳性急性混合细胞性白血病(Philadelphiachromosomepositivemixedacuteleukemia,Ph+MAL)13例。56例Ph+ALL中52例免疫表型为B细胞型。10例AML中,包括M14例,M2、M4和M7各2例。13例Ph+MAL中12例混合表达髓系和B淋巴细胞系表型,另1例为髓系、T淋巴细胞系混合型。总的染色体附加异常检出率为54.4%,附加异常较多涉及到的染色体包括:7号、双Ph染色体、+8等。Ph+ALL组和Ph+MAL组缓解率为57.0%,Ph+AML组无1例达到缓解。Ph+ALL完全缓解率明显低于同期正常核型ALL对照组(P<0.05)。Ph+ALL、Ph+MAL组总的中位生存期均为10个月。To explore the cytogenetics and related clinical characteristics of adult acute leukemia with Philadelphia chromosome positive (Ph+AL), MIC classification by morphology, immunology and cytogenetics was used to retrospectively study 79 patients with Ph+AL hospitalized in the Institute of Hematology, People Hospital in Beijing from October 1991 to September 2003. The results showed that 6.9% cases were diagnosed as Ph+AL and classified into three subtypes: acute lymphoblastic leukemia (Ph+ALL) in 56 patients (18%), acute myeloid leukemia (Ph+AML) in 10 patients (1.2%) and mixed acute leukemia (Ph+MAL) in 13 patients. B-cell antigen expression was found in 52 out of 56 patients with Ph+ALL. 54.4%(43/79) patients had additional chromosome abnormalities including chromosome 7, double Ph and plus 8, etc. Complete remission (CR) rate of Ph+ALL and Ph+MAL was 57.0%, none of Ph+AML achieved CR. Median overall survival of Ph+ALL, Ph+MAL and Ph+AML were 10, 10 and 2.5 months respectively. It is concluded that Ph+AL has highly heterogeneity involving various differentiated stages of immature leukemic cells. Since the poor prognosis associated with this kind of AL, early diagnosis with MIC classification is a prerequisite to take more effective conditioning regimen and prospectively consideration of allogeneic stem cell transplantation to improve prognosis.

关 键 词：Ph染色体阳性急性白血病 MIC分类 急性混合细胞性白血病 

分 类 号：R733.71[医药卫生—肿瘤]