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大多数婴儿白血病中存在两个以上免疫球蛋白重链J区基因。

More than two immunoglobulin heavy chain J region genes in the majority of infant leukemia.

作者信息

Rechavi G, Brok-Simoni F, Katzir N, Mandel M, Umiel T, Stark B, Zaizov R, Ben-Bassat I, Ramot B

机构信息

Institute of Hematology, Chaim Sheba Medical Centre, Tel-Hashomer, Israel.

出版信息

Leukemia. 1988 Jun;2(6):347-50.

PMID:3131595
Abstract

Congenital and infant leukemia are rare conditions associated with a very poor prognosis due to the high frequency of adverse clinical and laboratory parameters. As the occurrence of multiple immunoglobulin heavy chain hybridization band in childhood leukemia has been associated with poor prognosis, we studied whether it was present in this type of leukemia as well. Seven cases were examined, 4 of them less than 7 months of age. The immunophenotype was lymphoid in 5 and hybrid in 2. Most had abnormal karyotypes. In 5 of the 7, including all with congenital leukemia, an immunoglobulin heavy chain J region multiband pattern was found by Southern blot. The multiband pattern, whether primary or due to clonal evolution, seems to be associated with poor prognosis.

摘要

先天性和婴儿白血病是罕见疾病,由于不良临床和实验室参数的高频率出现,其预后非常差。由于儿童白血病中多重免疫球蛋白重链杂交带的出现与预后不良相关,我们也研究了这种情况是否存在于这类白血病中。共检查了7例,其中4例年龄小于7个月。免疫表型为淋巴细胞型的有5例,混合型的有2例。大多数病例有异常核型。在7例中的5例,包括所有先天性白血病患者,通过Southern印迹法发现免疫球蛋白重链J区多带模式。这种多带模式,无论是原发性的还是由于克隆进化导致的,似乎都与预后不良有关。

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