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目的 报告一例特殊的白血病病例具有M2 亚型的t(8;2 1) /AML1 ETO核型分子标志 ,却被全反式维甲酸单独诱导分化而获完全缓解 ,这提示全反式维甲酸还可能由其他通路介导髓系白血病细胞的分化。方法 一名 16岁的女孩于 1997年 9月 1日入院。根据FAB分型形态学初次诊断为M3型白血病。通过对骨髓新鲜细胞的免疫表型分析 ,R带显带技术分析细胞核型和体外诱导分化检测观察全反式维甲酸作用下骨髓原代细胞的分化程度。再以RT PCR扩增PML/RARα和AML1 ETO融合基因表达产物 ,以进一步确诊其亚型。在M3型白血病诊断作出后即给予口服ATRA 6 0mg/天 ,每周查血常规及血细胞分类记数两次。腰穿检查脑脊液。必要时给予抗菌素控制感染 ,并给予输液或输血支持。结果 对骨髓和外周血细胞以流式细胞术测免疫表型为CD13和CD33表面抗原阳性 ,表明属粒系。核型分析为女性核型 ,具有t(8;2 1) ,但并未发现t(15 ;17)。体外原代细胞能被ATRA诱导快速分化。在ATRA作用 4 8,72 ,96和 12 0小时后 ,NBT还原率阳性细胞的百分比分别达 4 0 %,79%,85 %和 93%。这一分化态势与NB4细胞完全相符。多次RT PCR扩增均不能发现长型或短型PML/RARα转录产物 ,却明白无误地测到AML1 ETO转录产物。据此对患者的诊断由M3 改为M2 。患者接受ATRA治?
PURPOSE: To report a case-specific t (8; 2 1) / AML1 ETO karyotype marker with a M2 subtype in a specific leukemia case completely relieved by all-trans retinoic acid alone, suggesting that all-trans retinoic acid Myeloid leukemia cells may be differentiated by other pathways. Method A 16-year-old girl was admitted on September 1, 1997. The first diagnosis of M3 leukemia according to FAB typing morphology. Immunophenotype analysis of bone marrow fresh cells, R banding banding analysis of cell karyotype and induced differentiation in vitro detection of all-trans retinoic acid-induced differentiation of bone marrow cells. The PCR products of PML / RARα and AML1 ETO fusion gene were amplified by RT PCR to further confirm their subtypes. Oral ATRA 60mg / day was given after diagnosis of type M3 leukemia. Blood routine and blood cell count were counted twice weekly. Waist wear check cerebrospinal fluid. Antimicrobials are given to control the infection if necessary and given infusion or transfusion support. Results The immunophenotypes of bone marrow and peripheral blood cells by flow cytometry were positive for CD13 and CD33 surface antigens, indicating that they belong to the maize lineage. Karyotype was female karyotype with t (8; 21), but no t (15; 17) was found. Primary cells in vitro can be rapidly differentiated induced by ATRA. After 48, 72, 96 and 120 hours of ATRA treatment, the percentages of NBT reduction-positive cells were 40%, 79%, 85% and 93%, respectively. This differentiation is in complete agreement with NB4 cells. Multiple RT PCR amplification failed to detect long or short PML / RARα transcripts but unambiguously detected AML1 ETO transcripts. According to the diagnosis of patients from M3 to M2. Patients undergoing ATRA treatment?