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与20号染色体缺失相关的血液系统疾病:107例患者的临床病理研究

Hematologic disorders associated with deletions of chromosome 20q: a clinicopathologic study of 107 patients.

作者信息

Kurtin P J, Dewald G W, Shields D J, Hanson C A

机构信息

Division of Hematopathology, Mayo Clinic, Rochester, Minnesota 55905, USA.

出版信息

Am J Clin Pathol. 1996 Nov;106(5):680-8. doi: 10.1093/ajcp/106.5.680.

Abstract

To study the pathogenetic significance of acquired del(20q), bone marrow morphology and clinical histories of 107 patients with hematologic disorders and the del(20q) were studied. In 84 cases representing myelodysplastic syndromes (47), myeloproliferative disorders (31), acute myeloid leukemia (3), pure red blood cell aplasia (2), and angioimmunoblastic lymphadenopathy with dysproteinemia (AILD-like T-cell lymphoma (1), 20q- was the sole karyotypic abnormality. The breakpoints on chromosome 20 were not constant, and in 77% of cases, only a fraction of the studied metaphases had del(20q). In 23 cases, including myelodysplastic syndromes (13), myeloproliferative disorders (6), acute myeloid leukemia (2), and autoimmune disorders (2), 20q- occurred with other cytogenetic abnormalities, including del(5q), -7, +8, 13q deletion or translocation, and +21. Despite the diagnostic heterogeneity, the bone marrow morphologic abnormalities consistently involved the erythroid precursors and megakaryocytes. 20q- is a primary cytogenetic abnormality occurring in hematologic disorders unified morphologically by dysplasia in erythroid precursors and megakaryocytes. In conjunction with other studies of disorders involving del(20q) or genes on the long arm of chromosome 20, the findings suggest that del(20q) disproportionately affects a common megakaryocytic/RBC stem cell.

摘要

为研究获得性20号染色体长臂缺失(del(20q))的致病意义,我们对107例血液系统疾病伴del(20q)患者的骨髓形态学和临床病史进行了研究。在84例患者中,包括骨髓增生异常综合征(47例)、骨髓增殖性疾病(31例)、急性髓系白血病(3例)、纯红细胞再生障碍性贫血(2例)以及伴有异常蛋白血症的血管免疫母细胞性淋巴结病(AILD样T细胞淋巴瘤,1例),del(20q)是唯一的核型异常。20号染色体上的断点并不固定,77%的病例中,仅部分研究的中期细胞存在del(20q)。在23例患者中,包括骨髓增生异常综合征(13例)、骨髓增殖性疾病(6例)、急性髓系白血病(2例)和自身免疫性疾病(2例),del(20q)与其他细胞遗传学异常同时出现,包括del(5q)、-7、+8、13号染色体长臂缺失或易位以及+21。尽管诊断存在异质性,但骨髓形态学异常始终累及红系前体细胞和巨核细胞。del(20q)是一种原发性细胞遗传学异常,在血液系统疾病中出现,其形态学特征为红系前体细胞和巨核细胞发育异常。结合其他关于涉及del(20q)或20号染色体长臂上基因的疾病研究,这些发现提示del(20q)对常见的巨核细胞/红细胞干细胞影响尤为明显。

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