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急性白血病中的13三体综合征。

Trisomy 13 in acute leukemia.

作者信息

Baer M R, Bloomfield C D

机构信息

Department of Medicine, Roswell Park Cancer Institute, Buffalo, New York 14263.

出版信息

Leuk Lymphoma. 1992 May;7(1-2):1-6. doi: 10.3109/10428199209053596.

Abstract

Trisomy 13 occurring as a sole cytogenetic abnormality has recently been demonstrated to have adverse prognostic significance in acute leukemia. Trisomy 13 is seen primarily in an older male population, and has been reported in treatment-associated acute leukemia and acute leukemia evolved from myelodysplastic syndromes, as well as in de novo leukemia. The 36 cases of acute leukemia with trisomy 13 reported to date include 26 AML, 6 AUL, 2 ALL and 2 mixed lineage patients. Immunophenotyping studies have demonstrated an undifferentiated phenotype or biphenotypic markers in most cases. Trisomy 13 is associated with a low complete remission rate and with brief remission duration. The role of the additional copy of chromosome 13 in the pathogenesis of these cases of acute leukemia and the gene(s) of importance on chromosome 13 are yet to be determined.

摘要

作为唯一细胞遗传学异常出现的13号染色体三体最近已被证明在急性白血病中具有不良预后意义。13号染色体三体主要见于老年男性人群,在治疗相关急性白血病、由骨髓增生异常综合征演变而来的急性白血病以及原发性白血病中均有报道。迄今为止报道的36例伴有13号染色体三体的急性白血病病例包括26例急性髓系白血病(AML)、6例急性未分化白血病(AUL)、2例急性淋巴细胞白血病(ALL)和2例混合谱系患者。免疫表型研究表明,大多数病例具有未分化表型或双表型标志物。13号染色体三体与完全缓解率低和缓解期短有关。13号染色体额外拷贝在这些急性白血病病例发病机制中的作用以及13号染色体上重要的基因尚未确定。

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