Zhao Jie, Yin Yu-Ming, Zhao Yan-Li, Sun Yuan, Wang Jing-Bo, Zhong Jing, Zhang Xian, Fei Xin-Hong, Shan Fu-Xiang, Liu Hong-Xing, Wang Tong, Wang Hui, Tong Chun-Rong, Wu Tong, Lu Dao-Pei
Beijing Daopei Hospital, Beijing 100049, China.
Zhongguo Shi Yan Xue Ye Xue Za Zhi. 2010 Dec;18(6):1381-5.
This study was aimed to analyze the clinical and cytogenetic characteristics of acute leukemia with 11q23/mll rearrangement and explore the reasonable therapeutic principles. Characteristics in general situation, morphology, immunology, molecular biology, cytogenetics, treatment and overall survival of 36 cases of acute leukemias with mll gene rearrangement were studied and analyzed. The results showed that 36 cases with mll gene rearrangement were found positive (7.2%) in 494 patients with acute leukemia. Among the 36 cases of mll rearrangement positive, 32 cases were diagnosed as acute myeloid leukemia (AML) with myeloid antigen expression, of which 5 cases expressed lymphoblastic differentiation antigen; 4 cases were classified as B-lineage acute lymphoblastic leukemia (ALL), of which non-lineage myeloid expression pattern were found in 3 cases. In 29 out of 36 cases (80%) the clonal chromosomal aberration were detected, of which chromosome 11 aberration were observed in 22 cases. All patients received chemotherapy with a total response rate of 47.2%. Of the responded patients, 10 cases relapsed within 6 months, with a recurrence rate of 40%; 9 cases received hematopoietic stem cell transplantation (HSCT), 7 cases of which survived after transplantation. The median survival time of 36 cases was 16 months (range 2 - 46) and their 2-year overall survival rate was 41.4%. The 2-year overall survival rate of 9 patients who received HSCT was 87.5%. It is concluded that acute leukemia patients with mll gene rearrangement show poor response to chemotherapy, high recurrence rate and poor prognosis. Hematopoietic stem cell transplantation may be a reasonable treatment principle to improve these patients' survival situation.
本研究旨在分析伴11q23/mll重排的急性白血病的临床及细胞遗传学特征，并探索合理的治疗原则。对36例mll基因重排的急性白血病患者的一般情况、形态学、免疫学、分子生物学、细胞遗传学、治疗及总生存情况进行研究分析。结果显示，在494例急性白血病患者中，36例mll基因重排者呈阳性（7.2%）。36例mll重排阳性患者中，32例诊断为伴有髓系抗原表达的急性髓系白血病（AML），其中5例表达淋巴细胞分化抗原；4例归类为B系急性淋巴细胞白血病（ALL），其中3例发现有非系髓系表达模式。36例中有29例（80%）检测到克隆性染色体畸变，其中22例观察到11号染色体畸变。所有患者均接受化疗，总缓解率为47.2%。缓解患者中，10例在6个月内复发，复发率为40%；9例接受造血干细胞移植（HSCT），其中7例移植后存活。36例患者的中位生存时间为16个月（范围2 - 46个月），2年总生存率为41.4%。9例接受HSCT患者的2年总生存率为87.5%。结论是，mll基因重排的急性白血病患者对化疗反应差、复发率高、预后不良。造血干细胞移植可能是改善这些患者生存状况的合理治疗原则。
