急性双表型白血病的临床及生物学特征研究

Study on the clinical characteristics of biphenotypic acute leukemia identified

目的分析急性双表型白血病(BAL)的临床、生物学特征及治疗疗效。方法对40例急性双表型白血病患者的临床和实验室资料,应用流式细胞术进行免疫分析,采用直接培养法或24/48h短期培养法G显带技术检测染色体。应用兼顾髓系和淋系的V(M)DALP方案联合化疗,Ph阳性患者部分加用伊马替尼治疗。结果符合急性双表型白血病诊断的40例,其中男26例,女14例,发病时WBC≥30×109/L者20例(50.0%),WBC≥100×109/L者6例(15.0%),合并肝、脾肿大者16例(40.0%),2例患者(5.0%)合并中枢神经系统白血病;以同时表达髓系和B系抗原者最常见,占72.5%,髓系和T系抗原共同表达者占25.0%,B系和T系抗原共同表达者占2.5%。急性双表型白血病患者中CD34阳性高表达占80.0%;对30例患者进行了染色体核型分析,正常核型者13例,占43.3%,异常核型者17例,占56.7%,其中9例(30.0%)Ph染色体阳性表达;40例患者完全缓解19例(47.5%),9例Ph染色体阳性患者完全缓解3例(33.3%)。结论急性双表型白血病高白细胞者多见,以表达髓系和B系抗原共表达者最常见,异常核型发生率高,Ph染色体阳性者多见,治疗效果较差,预后不良。

Objective To analyze the clinical and biological characteristics and prognosis of biphenotypic acute leukemia（BAL）.Methods Immunophenotypes were analyzed using multicolor flow cytometry,karyotype analysis by short-term culture G-banding technique.The chemotherapy regimens were accordingly for both acute lyphoblastic leukaemia（ALL）,acute myeloid leukaemia（AML）.Patients with Ph（＋）were treated with Imatinib.Results 50.0% of the BAL patients had WBC ≥30×109/L and 15.0% WBC≥100×109/L,Percentages of coexpression of myeloid and B lymphoid antigens were 72.5%,of myeloid and T lymphoid antigens 25.0%,and of B and T lymphoid antigens was 2.5%.The rate of CD34 antigen expression was 80.0% in BAL.Normaland abnormal karyotypes accounted for 43.3%and 56.7%,respectively in 40 BAL patients with karyotype analysis.The rate of Ph（＋）was 25.0%.19（47.5%）of 40 patients achieved complete remission（CR）,but CR rate was only 33.3% for Ph（＋）cases.Conclusion High white blood cell count and coexpression of myeloid and B lymphoid antigens are common in BAL.Abnormal karyotype and Ph（＋）often happen.The treatment outcome of BAL is poor.