Lu Jing, Ashwani Neetika, Zhang Mingying, He Hailong, Lu Jun, Wang Yi, Zhao Wenli, Cao Lan, Ji Zhenghua, He Yaxiang, Hunag Yiping, Chen Ruihua, Hu Shaoyan
Department of Hematology and Oncology, Children's Hospital of Soochow University, Suzhou, 215003 China.
Institute of Clinical Immunology, The First Affiliated Hospital of Soochow University, Suzhou, China.
Indian J Hematol Blood Transfus. 2015 Mar;31(1):32-7. doi: 10.1007/s12288-014-0372-6. Epub 2014 Apr 12.
Mixed phenotype acute leukemia (MPAL) is a rare type of acute leukemia with a poor clinical outcome which lacks specific therapy. To evaluate the therapeutic efficiency of CCLG-2008 protocol used for acute lymphoblastic leukemia (ALL) in China on MPAL children who were initially diagnosed as ALL by morphology, we reviewed patients' database diagnosed as ALL and MPAL according to WHO classification and compared their outcomes from July 2008 to June 2012. Total newly enrolled ALL in this study were 309 cases by morphology, in which ten cases were identified as MPAL mainly by immunophenotyping: B+ myeloid (3/10), T+ myeloid (2/10), B + T (4/10), trilineage (1/10). Two cases were classified as intermediate risk (IR) and 8 cases were high risk (HR) according to the CCLG-2008 criteria. Only one case of IR survived and others died due to primary resistance of chemotherapy and relapse. Compared with MPAL, ALL children in IR and HR had a longer survival (28.1 vs 9.5 months, p < 0.0001) and lower relapse (16.3 vs 85.7 %, p = 0.0002). In a summary, our result indicated that MPAL in children is a poor-risk disease which needs personalized therapy to improve outcome.
混合表型急性白血病(MPAL)是一种罕见的急性白血病,临床预后较差,且缺乏特异性治疗方法。为评估中国用于急性淋巴细胞白血病(ALL)的CCLG - 2008方案对初诊为ALL但经形态学诊断为MPAL的儿童患者的治疗效果,我们回顾了2008年7月至2012年6月期间根据WHO分类诊断为ALL和MPAL的患者数据库,并比较了他们的治疗结果。本研究中通过形态学新入组的ALL患者共309例,其中10例主要通过免疫表型鉴定为MPAL:B + 髓系(3/10)、T + 髓系(2/10)、B + T(4/10)、三系(1/10)。根据CCLG - 2008标准,2例被分类为中危(IR),8例为高危(HR)。IR组仅1例存活,其他患者因化疗原发耐药和复发死亡。与MPAL相比,IR和HR组的ALL儿童生存期更长(28.1个月对9.5个月,p < 0.0001),复发率更低(16.3%对85.7%,p = 0.0002)。总之,我们的结果表明儿童MPAL是一种高危疾病,需要个性化治疗以改善预后。