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毛细胞白血病患者的细胞遗传学研究

Cytogenetic studies in patients with hairy cell leukemia.

作者信息

Ueshima Y, Alimena G, Rowley J D, Golomb H M

出版信息

Hematol Oncol. 1983 Jul-Sep;1(3):215-26. doi: 10.1002/hon.2900010304.

Abstract

We performed cytogenetic studies on 58 patients with hairy cell leukemia (HCL) from 1975 to 1981. Analysable metaphase cells stained with Q-banding were obtained in 77 samples from 44 patients. Cells with abnormal chromosomes were found in both unstimulated and stimulated cultures of bone marrow and peripheral blood. Patients were classified in 6 groups. Group I, 2 patients with a clonal chromosome abnormality; group II, 13 patients with nonclonal structural abnormalities; group III, 5 patients with nonclonal numerical abnormalities; group IV, 19 patients with only a normal karyotype; group V, 15 patients with no or with fewer than 5 normal metaphase cells; group VI, 4 patients with questionable abnormal chromosomes. Common abnormalities were deletion of the long arm of No. 6 or +3 each in 3 patients, and +Y, +12 or +18 in 2 patients. Actuarial survival for each group was calculated from diagnosis and also from chromosome examination. The two patients with a clonal chromosome abnormality died within one year. Eight of 13 patients with nonclonal structural abnormalities died within 5 years after diagnosis, while none of 5 patients with nonclonal numerical abnormalities and 2 of 19 patients with normal chromosomes died within 5 years. The difference in the 5-year actuarial survival between patients with nonclonal abnormalities (groups II and III) and those with a normal karyotype was significant (p less than 0.05). The difference was more marked between patients with nonclonal structural abnormalities and those with a normal karyotype (p less than 0.01). Patients with nonclonal numerical abnormalities had a longer survival than those patients with nonclonal structural abnormalities (p less than 0.05). Thus, structural chromosome abnormalities in HCL may be a poor prognostic sign even when they are not clonal.

摘要

1975年至1981年期间,我们对58例毛细胞白血病(HCL)患者进行了细胞遗传学研究。从44例患者的77份样本中获得了经Q带染色且可分析的中期细胞。在骨髓和外周血的未刺激及刺激培养物中均发现了染色体异常的细胞。患者被分为6组。第一组,2例有克隆性染色体异常;第二组,13例有非克隆性结构异常;第三组,5例有非克隆性数目异常;第四组,19例核型仅正常;第五组,15例无或正常中期细胞少于5个;第六组,4例染色体异常情况存疑。常见异常包括3例患者6号染色体长臂缺失或 +3,2例患者 +Y、+12或 +18。从诊断时以及染色体检查时开始计算每组患者的精算生存率。2例有克隆性染色体异常的患者在1年内死亡。13例有非克隆性结构异常的患者中有8例在诊断后5年内死亡,而5例有非克隆性数目异常的患者和19例核型正常的患者中在5年内均无死亡。非克隆性异常患者(第二组和第三组)与核型正常患者之间的5年精算生存率差异有统计学意义(p小于0.05)。非克隆性结构异常患者与核型正常患者之间的差异更为显著(p小于0.01)。有非克隆性数目异常的患者比有非克隆性结构异常的患者生存期更长(p小于0.05)。因此,HCL中的染色体结构异常可能是一个不良预后指标,即使它们不是克隆性的。

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