慢性髓细胞白血病急变期MICM分型回顾性分析

Analysis of Morphology,Immunology,Cytogentic and Molecular Biology Classification of Patients with Chronic Myeloid Leukemia in Blastic Crisis

目的:探讨慢性髓细胞白血病急变期(CML-BC)患者的细胞形态学(M)、免疫学(I)、细胞遗传学(C)和分子生物学(M)的特征及应用价值。方法:对38例CML-BC患者的MICM分型进行回顾性分析。结果:以FAB分型为基础的形态学确诊率达94.7%;免疫分型结果为:38例CML-BC中CML-AML占71.0%,其中37.0%伴淋系表达;CML-ALL占23.7%,均为B细胞性,其中66.67%伴髓系表达;CML-MAL(混合性白血病)占5.3%,均为B系和髓系混合表达;CD34+26例(68.4%),CD7+10例(26.3%),均与CD34共表达。细胞遗传学结果显示:CML特征性Ph染色体检出率为94.3%(36/38),附加异常染色体检出率为60.5%(23/38),发生频率较高的类型是+Ph、+8和i(17q);FISH检测BCR/ABL融合基因阳性率为100%,der(9)缺失占14.7%。RT-PCR检测20例患者BCR/ABL融合基因均为阳性,其中b2a2型(12/20),b3a2型(8/20),1例(1/20),b2a2和b3a2双阳性(1/20)。结论:CML-BC是造血干细胞疾病,原始细胞分化阻滞在早期阶段,预后差。MICM分型对CML-BC的诊断、治疗和预后判断均有重要价值。

Objective： To invistigate the characteristic and value of bone marrow morphology, immunology, cytogenetic and molecular biology （MICM） in patients with chronic myeloid leukemia in blastic crisis （CML-BC）. Methods： 38 patients with CML-BC were studied by MICM analysis. Results： The accuracy rate of morphological diagonis based on FBA classification was 94.7%. Immunology results, the percentage of CML-AMLwas 71.0%, in which 37.0% patients expressed lympholytic associated antigen; The percentage of CML-ALL was 23.7% and all were B lypholytic immunophenotype, in which 66.7% patients expressed myeloid assuiated antigen; The percentage of CML-MALwas 5.3% with all expressing B lypholoid and myeloid immumphenotype. 26 cases （68.4%） had CD34 expression and 10 cases （26.3%） had CD7 and CD34 Co-expression. In cytogenetic result, the positive rate of Ph chromosome was 94.3%. Other chromosome abnomality rate was 60.5% with ＋ph,＋8 and i （17q） in high appearance rate. The positive rate of BCR/ABL gene by FISH reached 100% with 14.7% del（9）. The positive rate ofBCR/ABLgene by RT-PCR also reached 100% with b2a3（12/20）,b3a2（8/20） and b2a3＋b3a2（1/20）. Conclusion： CML-BC is a kind of disease of stem cell. The differentiation of blast cell is blocked in the early stage. MICM has great value in diangnosis, therapy and prognosis judgement.