Ph染色体阳性急性白血病细胞遗传学及临床研究

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,Ph AML

Cytogenetic and Clinical Study of Philadelphia Chromosome Positive Adult Acute Leukemia

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.