160例急性淋巴细胞白血病MICM分型及其治疗和预后相关性的研究

MICM classification and its correlation with therapy and prognosis in childhood actue lymphoblastic leukemia

目的 研究儿童急性淋巴细胞白血病 (ALL)的MICM分型及其与治疗、预后的相关性。方法 采用细胞形态学检查、染色体R带或G带核型分析、流式细胞仪细胞免疫表型检测和套式逆转录聚合酶链反应 (RT PCR)检测TEL AML1、BCR ABL融合基因转录本。结果 在 16 0例儿童ALL中 ,76例为L1型 ,73例为L2 型 ,5例为L3 型 ,6例不能分类。在核型分析的 15 1例中 ,75例 (4 9 7% )有克隆性染色体异常。包括超二倍体 16例 ,假二倍体 12例 ,亚二倍体 2 6例 ,染色体易位 2 1例。后者包括t(4 ;11) 7例 ,t(9;2 2 ) 6例 ,其他少见易位 8例。进行过免疫表型分析的 12 8例中 ,6 4例 (5 0 0 % )为B系表达 ,38例 (2 9 7% )为T系表达 ,14例 (10 9% )为T、B混合表达 ,6例为B系、髓系混合表达 ,3例为T系、髓系混合表达 ,其他 3例。套式RT PCR检测到TEL AML1融合基因转录本 11例、BCR ABL融合基因转录本 2例。 12 5例ALL患儿分别接受DOLP(柔红霉素、长春新碱、左旋门冬酰胺酶、泼尼松或地塞米松 ) ,CODP(环磷酰胺、长春新碱、柔红霉素、泼尼松或地塞米松 )或COLP(环磷酰胺、长春新碱、左旋门冬酰胺酶、泼尼松或地塞米松 )方案治疗 ,其中 116例 (92 8% )获得完全缓解 (CR)。CR达 2年以上者 16例 ,1年以上者 2 8例 ,6个月以上者 17例。?

Objective To investigate the correlation among MICM (morphologic, immunologic, cytogenctic and molecular) classification, chemotherapy and prognosis of childhood actue lymphoblastic leukemia (ALL). Methods Slides with Wright stain were examined for cell morphology. Karyotype was analyzed by R-or G-banding technigue. Cell immunophenotype was determined by flow cytometry. The nest RT-PCR was performed to detect TEL-AML1 and BCR-ABL fusion gene transcript. Results Among 160 childhood patients with ALL, 76 were diagnosed as ALL-L_1,73 as ALL-L_2, 5 as ALL-L_3, 6 unclassified. Karyotype analysis was performed in 151 patient. Seventy-five of them (49.7%) had clonal chromosomal abnormalities, which consisted of hyperdiploidy (16 cases), hypodiploidy (26 cases), pseudodiploidy (12 cases) and chromosome translocation (21 cases). Seven cases were found positive for 4;11 translocation, 6 cases for 9;22 translocation and 8 cases for other rare translocations. Immunophenotype analysis was carried out in 128 cases. Sixty-four cases (50.0%) expressed B-cell antigen, 38 cases (29.7%) T-cell antigen, 14 cases (10.94%) T and B antigens, 6 cases B and myeloid antigens, 3 cases T and myeloid antigens, 1 case lymphoid and myeloid antigens, 1 case myeloid antigen, 1 case T and megakaryocytic antigens. TEL-AML1 fusion gene transcript was detected in 11 cases and BCR-ABL fusion gene transcript in 2 cases. One hundred and twenty-five cases were treated with DOLP(DNR, VCR,L-ASP, Pred), CODP(CTX, VCR, DNR, Pred) or COLP(CTX, VSR, L-ASP, Pred) regimens, respectively, of whom, 116 cases (92.8%) obtained complete remission (CR) which lasted for more than two years in 16 cases, more than one year in 28 cases, more than six months in 17 cases. Conclusions It had little clinical significance that childhood ALL was morphologically classified as L1 or L2 subtypes; B-cell ALL is the major immunologic subset in childhood ALL; In childhood B-ALL, the prognosis was the best for those with hyperdiploidy or 12;21 translocation, medium for 4;11translocation, poor for 9;22 translocation. In childhood ALL patients whose T or B immunophenotype distribution had no difference, the prognosis of those with pseudodiploidy was better than those with hypodiploidy or normal diploidy;MICM classification was more accurate and perfect than FAB criteria because it could shed light on the essence of ALL. Moreover, it has important value for guiding the treatment of ALL so that the rates of CR and long-term survival of patients with ALL would be improved.