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10号染色体三体综合征的存活情况:文献综述及7例新病例报告

Trisomy 10 survival: a literature review and presentation of seven new cases.

作者信息

Pedersen B, Andersen C L, Søgaard M M, Nørgaard J M, Koch J, Krejci K, Brandsborg M, Clausen N

机构信息

Department of Cytogenetics, Danish Cancer Society, Aarhus, Denmark.

出版信息

Cancer Genet Cytogenet. 1998 Jun;103(2):130-2. doi: 10.1016/s0165-4608(97)00387-7.

Abstract

Trisomy 10 as the only chromosome aberration is a rare phenomenon in malignant and premalignant hemopoietic disorders. We describe 7 new cases and have found another 12 in the literature. It appears that, whereas adult patients have myeloid disorders (acute myeloid leukemia, myeloproliferative, or myelodysplastic syndromes), in children the diagnosis is lymphocytic leukemia or lymphoma. The median survival was 122 months in the total material. Age above 60 years proved to be a significant adverse factor (median survival only 5 months; p = 0.003). None of the other clinical, cytogenetic, or hematological variables were of demonstrable prognostic importance. In contrast with the larger trisomy 10 clones, those of limited size were associated with nonleukemic diagnoses, normal or slightly elevated leukocyte counts, and few or no circulating blasts. This may suggest that expansion of the trisomy 10 clone is associated with clinical and hematological progression.

摘要

10号染色体三体作为唯一的染色体畸变在恶性和癌前造血系统疾病中是一种罕见现象。我们描述了7例新病例,并在文献中又发现了12例。似乎成年患者患有髓系疾病(急性髓系白血病、骨髓增殖性疾病或骨髓增生异常综合征),而儿童的诊断为淋巴细胞白血病或淋巴瘤。全部病例的中位生存期为122个月。60岁以上被证明是一个显著的不利因素(中位生存期仅5个月;p = 0.003)。其他临床、细胞遗传学或血液学变量均无明显的预后意义。与较大的10号染色体三体克隆不同,那些规模有限的克隆与非白血病诊断、白细胞计数正常或略有升高以及很少或没有循环原始细胞相关。这可能表明10号染色体三体克隆的扩增与临床和血液学进展有关。

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