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13三体综合征:一种优先影响男性的染色体畸变,特异性干扰髓系增殖和分化?一例报告及文献综述

Trisomy 13: a preferentially male chromosome aberration interfering specifically with myeloid proliferation and differentiation? Report of a case and review of the literature.

作者信息

Pedersen B, Jensen I M

机构信息

Danish Cancer Society, Department of Cytogenetics, Aarhus Amtssygehus.

出版信息

Cancer Genet Cytogenet. 1991 Nov;57(1):79-85. doi: 10.1016/0165-4608(91)90192-w.

Abstract

A case of trisomy 13 is presented: a 73-year-old man with acute nonlymphocytic leukemia (ANLL), FAB borderline M1/M2, and peripheral leukocyte and platelet counts that were difficult to control with chemotherapy. A literature review shows that 35 cases of trisomy 13 are known at present. They are characterized by male predominance (76%), preferentially myeloid disorders (ANLL, myelodysplastic syndromes, chronic myeloid leukemia), leucocytosis, and relatively high platelet counts and hemoglobins. It is suggested that trisomy 13 is a specific nosologic entity with male predominance and characterized by interference with proliferation and differentiation in the myeloid differentiation series.

摘要

本文报告一例13三体综合征病例:一名73岁男性,患有急性非淋巴细胞白血病(ANLL),FAB分型为临界M1/M2,外周血白细胞和血小板计数难以通过化疗控制。文献综述显示,目前已知35例13三体综合征病例。其特征为男性占优势(76%),主要为髓系疾病(ANLL、骨髓增生异常综合征、慢性髓系白血病),白细胞增多,血小板计数和血红蛋白相对较高。提示13三体综合征是一种以男性占优势为特征的特定疾病实体,其特点是干扰髓系分化系列中的增殖和分化。

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