Clinical And Laboratorial Characteristics Analysis For 15 Cases Of Acute Myeloid Leukemia With Ph Chromosome/Positive /BCR-ABL

Background and ObjectiveThe Ph chromosome is formed by the mutual translocation of the abl gene on the long arm of chromosome 9（9q34）with the bcr gene on the long arm of chromosome 22(t（9;22）（q34;q11）.The basis of molecular biology is the fusion of the BCR-ABL gene and is a hallmark of chronic myeloid leukemia（CML）.About95%of CML patients have the Ph chromosome and are also found in acute leukemias,with the in-depth development of laboratory detection technology,Ph chromosomes can also be detected in acute leukemia（AL）.And the Ph chromosome positive rates in children and adults are 2%-10%and 10%-30%,respectively,in acute lymphoblastic leukemia,whereas Ph chromosome A positive acute myeloid leukemia（Ph⁺AML）Incidence is low（0.9%-3%）,in recent years the literature reported a lower incidence of 0.45-1.0%,the rapid progress of such leukemia,induced remission rate,high recurrence rate,short survival,clinical reports Less and has unique clinical and biological characteristics.Tyrosine kinase inhibitor（TKI）mainly blocks the phosphorylation of BCR-ABL fusion gene and stem cell receptor factor,and has achieved remarkable results in the treatment of CML patients.At present,TKI has been applied to the Ph chromosome Of AML patients in this study by exploring the Ph chromosome and/or BCR-ABL clinical and laboratory features（AML）patients fusion gene-positive acute myeloid leukemia,the clinical characteristics of the disease,treatment and prognosis.MethodsRetrospectively analyzed the cell morphology,cytogenetics and cellular immunophenotyping of 15 diagnosed Ph chromosome positive AML patients in the First Affiliated Hospital of Zhengzhou University from January 2011 to January2017.Results（1）The detection rate of Ph/BCR-ABL⁺AML in the same period of AML in our hospital was 1.47%（15/1018）.The Results showed that 15patients（8 males and 7females）demonstrated Ph Chromosome and BCR-ABL Positive,their median age was 38（range 5-58）years old,median WBC count 38×10⁹/L（4.2-207.4×10⁹/L）and6 cases were extramedullary infiltration（40%,6/15）.（2）Based on FAB classification,M2（53.3%）,M4（26.7%）.Immnologic analysis of 15 Ph⁺AML patients showed that 3 cases were immunophenotyped with myeloid and lymphoid cell antigens,and the remaining 12 expressed myeloid cell antigens and all 15 cases expressed CD34.（3）The 15 cases all had chromosomal examination in the initial diagnosis,which can be detected with Ph positive chromosome,and some chromosomes were associated with additional chromosomal abnormalities.BCR-ABL fusion gene transcript positive(P190^BCR-ABLCR-ABL 26.7%,P210^BCR-ABL73.3%)were detected in all cases,among which 1 cases coexpressed CBFβ-MYH11.（4）4 out of 8 cases existed AML-like gene mutations（50%）.9 of 15 patients were completely relieved（60%,9/15）after one course of induction therapy.（5）The CR rate（85.7%,6/7）in combination with tyrosine kinase inhibitor（TKI）was better than chemotherapy alone（37.5%,3/8）,but the difference was not statistically significant（P=0.12）.Among all 15 patients,allogeneic hematopoietic stem cell transplantation（allo-HSCT）was performed in 4 patients,and the transplantation group was superior to the non-transplant group in the treatment.The differencewasstatisticallysignificantbetweenthetwogroups（P=0.032）.Chemotherapy combined with TKI group（13 months）and chemotherapy combined with HSCT group（60 months）were better than chemotherapy alone（8months）（P=0.0064;P=0.04）.At the end of the follow-up,chemotherapy combined with TKI and HSCT groups survived.Conclusions1.There are special characteristics of Ph chromosome/BCR-ABL gene positive AML in the clinical features,and peripheral blood white blood cells are often relatively high and easy to combine with extramedullary infiltration.2.There are poor prognosis of Ph/BCR-ABL⁺AML,and high expression of CD34 may be one of the characteristics of immune expression of Ph/BCR-ABL⁺AML.3.Ph/BCR-ABL⁺AML with additional chromosomal abnormalities may affect the survival of patients.4.The traditional chemotherapy regimens of Ph/BCR-ABL⁺AML have poor efficacy,easy to relapse,and poor efficacy and short survival time of Ph/BCR-ABL⁺AML implied that chemotherapy combined with TKI and followed by allo-HSCT in CR was the effective way to improve prognosis.