Dang R K, Anthony R S, Craig J I, Parker A C
Department of Haematology, Western General Hospital, Edinburgh, Scotland.
Hum Mutat. 1999;13(2):172. doi: 10.1002/(SICI)1098-1004(1999)13:2<172::AID-HUMU17>3.0.CO;2-N.
We report a novel p53 insertion in a case of aggressive acute lymphoblastic leukaemia in a 16 year old male, in which 2 separate leukaemic clones were previously identified by T-cell receptor sigma and immunoglobulin heavy chain gene rearrangement studies. Initial p53 mutation screening of blast cells from 29 patients with acute leukaemia by PCR-denaturing gradient gel electrophoresis showed that 2 had a silent codon 213 polymorphism and only the index case had a somatic mutation identified to be an 8 bp insertion in codon 281 (5'CCGGGGGG-3'). This insertion was associated with the second, more aggressive clone which underwent clonal evolution and became resistant to cytotoxic chemotherapy. With an allele-specific primer in PCR, we were able to demonstrate the presence of this clone as a minority at disease presentation, and in 2 of 3 collections of peripheral blood progenitor cells.
我们报告了一例16岁男性侵袭性急性淋巴细胞白血病患者中一种新的p53插入突变,此前通过T细胞受体σ和免疫球蛋白重链基因重排研究鉴定出两个不同的白血病克隆。通过PCR-变性梯度凝胶电泳对29例急性白血病患者的原始细胞进行初始p53突变筛查,结果显示2例有密码子213沉默多态性,只有该索引病例有一个体细胞突变,经鉴定为密码子281处8 bp插入(5'CCGGGGGG-3')。这种插入与第二个更具侵袭性的克隆相关,该克隆经历了克隆进化并对细胞毒性化疗产生耐药性。通过PCR中的等位基因特异性引物,我们能够证明该克隆在疾病初发时作为少数克隆存在,并且在外周血祖细胞的3次采集样本中有2次出现。