儿童急性淋巴细胞白血病IG/TCR重排的特点及临床意义

Characteristics and clinical significance of IG/TCR rearrangement in children with acute lymphoblastic leukemia

目的探索急性淋巴细胞白血病(ALL)患儿免疫球蛋白基因(IG)和T细胞受体基因(TCR)重排特点及其在白血病危险度分层中的作用。方法收集189例ALL患儿的骨髓标本及临床资料,采用BIOMED-2引物行多重PCR方法检测患儿IG/TCR重排,并进行统计分析。结果 189例ALL患儿中176例携带IG/TCR重排,其中男102例、女74例;IGVH重排阳性136例(77. 3%),IGDH阳性46例(26. 1%),IGK阳性92例(52. 3%),IGλ阳性11例(6. 3%),TCRB阳性47例(26.7%),TCRD阳性77例(43.8%),TCRG阳性88例(50.0%);25例患儿(14.2%)的IG/TCR重排仅有1个克隆,41例(23.3%)含2个克隆,61例(34.7%)含3个克隆,49例(27.8%)含3个以上克隆。与中危和标危相比,高危患儿中IGK阳性的比例较高,且具有统计学意义(χ~2=7.48,P=0.024);其他类型的IG/TCR重排与白血病的危险度无相关性(P>0.05)。结论 ALL患儿中IG/TCR重排普遍存在,IGK重排与ALL患儿的危险度相关。

ObjectiveTo investigate characteristics and clinical signifcance of IG/TCR rearrangement in children with acute lymphoblastic leukemia （ALL）. MethodsBone marrow samples and clinical data of 189 children with ALL were collected, and the IG/TCR rearrangements of patients were detected by multiplex polymerase chain reaction according to the method provided by BIOMED-2. ResultsIn our study, 176 children with ALL had IG/TCR rearrangement （102 male and 74 female）. 136 cases （77.3%） has IGVH rearrangement and 46 ALL patients （26.1%） has a IGDH rearrangement. IGK rearrangement was detected in 92 patients （52.3%）, and IGλ was detected in 11 patients （6.3%）. 47 cases （26.7%） has TCRB rearrangement, and 77 cases （43.8%） have TCRD rearrangement. TCRG rearrangement was detected in 88 patients （50.0%）. About 25 patients have only one clone of the IG/TCR rearrangement, about 41 patients have two clones of IG/TCR rearrangement, 61 patients have three clones, and 49 patients have more than three clones. We found IGK positive rate was higher in patients with high risk leukemia than that of medium risk and standard risk group , and the difference was statistically signifcant （ χ2=7.475, P=0.024）, and other IG/TCR rearrangement has no association with risk degree in ALL patients （P〉0.05）. ConclusionsThe rearrangement of IG/TCR in children with ALL is ubiquitous, and the IGK rearrangement is associated with the risk of ALL in children.