Asou N
Second Department of Internal Medicine, Kumamoto University Medical School.
Rinsho Ketsueki. 1991 Jun;32(6):629-35.
We studied gene rearrangement and expression of immunoglobulin heavy (IgH) chain, T cell receptor (TCR) beta, gamma and delta chains in neoplastic T cells from patients with leukemia and lymphoma. Rearrangements of TCR beta and gamma chain genes were observed in most of T cell neoplasms. TCR delta chain gene rearrangements or deletions were detected in all 77 T cell neoplasms; 6 of 9 CD3- T cell neoplasms showed rearrangement, whereas biallelic deletion of TCR delta chain gene was the most common pattern in CD3+ T cell neoplasm (65 of 68 patients). One patient with CD3- T cell leukemia had TCR delta chain gene rearrangement with a germline configuration of TCR beta, gamma and IgH chain genes. TCR gamma and delta chain gene transcripts were detected in most of the CD3- T cell neoplasms, whereas mature TCR alpha and beta chain mRNA were demonstrated in the majority of the CD3+ T cell neoplasms. In 6 patients with CD7+ CD3- CD4- CD8- MPO- leukemia, only 2 patients had rearrangements and weak expressions of IgH, TCR gamma and delta chain genes. We also present two cases of double negative (CD3+ CD4- CD8-) leukemia; one is TCR gamma delta bearing LGL, the other is TCR alpha beta bearing ATL. These results suggest that most of T cell neoplasms preserve a pattern of genotypic and phenotypic expression reflecting their developmental pathways and differentiation levels of TCR bearing normal T cells.
我们研究了白血病和淋巴瘤患者肿瘤性T细胞中免疫球蛋白重链(IgH)、T细胞受体(TCR)β、γ和δ链的基因重排及表达情况。在大多数T细胞肿瘤中观察到了TCRβ和γ链基因的重排。在所有77例T细胞肿瘤中均检测到TCRδ链基因重排或缺失;9例CD3 - T细胞肿瘤中有6例出现重排,而TCRδ链基因双等位基因缺失是CD3 + T细胞肿瘤中最常见的模式(68例患者中的65例)。1例CD3 - T细胞白血病患者的TCRδ链基因重排,同时TCRβ、γ链和IgH链基因呈种系构型。在大多数CD3 - T细胞肿瘤中检测到TCRγ和δ链基因转录本,而在大多数CD3 + T细胞肿瘤中检测到成熟的TCRα和β链mRNA。在6例CD7 + CD3 - CD4 - CD8 - MPO - 白血病患者中,只有2例患者的IgH、TCRγ和δ链基因发生重排且表达较弱。我们还报告了2例双阴性(CD3 + CD4 - CD8 - )白血病病例;1例是带有TCRγδ的大颗粒淋巴细胞白血病,另1例是带有TCRαβ的成人T细胞白血病。这些结果表明,大多数T细胞肿瘤保留了一种基因型和表型表达模式,反映了它们的发育途径以及携带TCR 的正常T细胞的分化水平。