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[血液肿瘤中FGFR1基因异常的临床意义]

[Clinical Significance of FGFR1 Gene Abnormalities in Blood Tumors].

作者信息

Zhang Chun-Ling, Tang Gu-Sheng, Guo Meng-Qiao, Cheng Hui, Liu Ming-Dong, Yang Jian-Min, Gong Sheng-Lan

机构信息

Department of Hematology, First Affiliated Hospital of Naval Military Medical University. Institute of Hematology of Chinese PLA, Shanghai 200433, China.

Department of Hematology, First Affiliated Hospital of Naval Military Medical University. Institute of Hematology of Chinese PLA, Shanghai 200433, China,E-mail:

出版信息

Zhongguo Shi Yan Xue Ye Xue Za Zhi. 2020 Jun;28(3):983-988. doi: 10.19746/j.cnki.issn.1009-2137.2020.03.044.

Abstract

OBJECTIVE

To study the potential significance and clinical application of FGFR1 gene abnormality in the diagnosis, clinical features, pathological mechanism and treatment in hematological tumors.

METHODS

Clinical data of total of 29 patient with chromosome of 8 short arm (8P) abnormality who had more comprehensive medical history from 2013 to 2018 were collected. The karyotype analysis of bone marrow chromosomes in patients was carried out by using chromosome R band banding technique. FGFR1 gene was detected by using fluorescence in situ hybridization (FISH).

RESULTS

Seven cases of FGFR1 gene abnormalities were decteted, including 3 cases of FGFR1 gene amplification, 2 cases of translocation, and 2 cases of deletion. Five patients with FGFR1 gene amplification or deletion not accompaned with eosinophilia, moreover the chromosome was a complex karyotype with poor prognosis; Two cases of FGFR1 gene translocation were non-complex chromosomal translocation and one of which survived for 6 years after bone marrow transplantation, the other chromosome karyotype showed no rearrangement of 8 short arm. However, FGFR1 gene rearrangement was confirmed by FISH analysis, which was a rare insertional translocation.

CONCLUSION

FGFR1 gene amplification or deletion often occur in cases with complex karyotype, which not accompany eosinophilia, moreover have poor prognosis. The patients with FGFR1 gene translocation accompany eosinophilia which is consistent with the clinical characteristics of myeloid / lymphoid neoplasms with FGFR1 abnormality. Karyotype analysis combined with FISH method can improve the detection of abnormal clones.

摘要

目的

探讨成纤维细胞生长因子受体1(FGFR1)基因异常在血液系统肿瘤诊断、临床特征、病理机制及治疗中的潜在意义及临床应用。

方法

收集2013年至2018年期间29例具有较完整病史的8号染色体短臂(8p)异常患者的临床资料。采用染色体R带显带技术对患者骨髓染色体进行核型分析。采用荧光原位杂交(FISH)技术检测FGFR1基因。

结果

检测到7例FGFR1基因异常,其中3例为FGFR1基因扩增,2例为易位,2例为缺失。5例FGFR1基因扩增或缺失患者无嗜酸性粒细胞增多,且染色体为复杂核型,预后较差;2例FGFR1基因易位为非复杂染色体易位,其中1例骨髓移植后存活6年,另1例染色体核型显示8号短臂无重排。然而,FISH分析证实FGFR1基因重排,为罕见的插入易位。

结论

FGFR1基因扩增或缺失常见于复杂核型病例,无嗜酸性粒细胞增多,预后较差。FGFR1基因易位患者伴有嗜酸性粒细胞增多,与FGFR1异常的髓系/淋系肿瘤临床特征相符。核型分析联合FISH方法可提高异常克隆的检出率。

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