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TAL1基因改变在儿童T细胞急性淋巴细胞白血病和淋巴瘤中的临床意义

Clinical significance of TAL1 gene alteration in childhood T-cell acute lymphoblastic leukemia and lymphoma.

作者信息

Kikuchi A, Hayashi Y, Kobayashi S, Hanada R, Moriwaki K, Yamamoto K, Fujimoto J, Kaneko Y, Yamamori S

机构信息

Division of Hematology/Oncology, Saitama Children's Medical Center, Japan.

出版信息

Leukemia. 1993 Jul;7(7):933-8.

PMID:8321044
Abstract

The TAL1 gene is altered as a consequence of t(1;14)(p32;q11) found in T-cell acute lymphoblastic leukemia (ALL) and shows site specific recombination (tald rearrangement). We investigated TAL1 gene alterations in 39 children with T-cell ALL, in 32 with B-precursor ALL, in three with ALL with myeloid-associated antigen, and in 18 with T-non-Hodgkin's lymphoma (T-NHL). tald rearrangement was found in nine of 39 T-cell ALL patients using Southern blot analysis with a TAL1 gene probe. Polymerase chain reaction (PCR) products predicted from the sequences of the corresponding tald alleles were shown in all of these patients. In contrast, no rearranged band was observed in other kinds of leukemia or in T-NHL patients. All of these patients with tald rearrangement had CD1- CD2+ CD4- CD7+ CD10- pheno-type. Of these, seven were classified as stage I thymic differentiation, and eight have survived for three to 59 months remission. Four of seven patients investigated had normal karyotypes, which has been reported to be associated with a good prognosis in T-cell ALL. We conclude that tald rearrangement is restricted to T-cell ALL, for which it provides a useful clonal marker. Such patients with this rearrangement may constitute a subgroup of T-cell ALL with a good prognosis.

摘要

TAL1基因因在T细胞急性淋巴细胞白血病(ALL)中发现的t(1;14)(p32;q11)而发生改变,并显示出位点特异性重组(tald重排)。我们研究了39例T细胞ALL患儿、32例B前体ALL患儿、3例伴有髓系相关抗原的ALL患儿以及18例T非霍奇金淋巴瘤(T-NHL)患者的TAL1基因改变情况。使用TAL1基因探针进行Southern印迹分析,在39例T细胞ALL患者中有9例发现了tald重排。所有这些患者均显示出根据相应tald等位基因序列预测的聚合酶链反应(PCR)产物。相比之下,在其他类型的白血病或T-NHL患者中未观察到重排条带。所有这些发生tald重排的患者均具有CD1-CD2+CD4-CD7+CD10-表型。其中,7例被分类为I期胸腺分化,8例已缓解存活3至59个月。所研究的7例患者中有4例核型正常,据报道这与T细胞ALL的良好预后相关。我们得出结论,tald重排仅限于T细胞ALL,它为此提供了一个有用的克隆标志物。具有这种重排的此类患者可能构成T细胞ALL中预后良好的一个亚组。

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