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伴有插入衍生 BCR-ABL1 融合的慢性髓性白血病:通过 FISH 和核型相关性来重新定义复杂染色体异常可预测预后。

Chronic myeloid leukemia with insertion-derived BCR-ABL1 fusion: redefining complex chromosomal abnormalities by correlation of FISH and karyotype predicts prognosis.

机构信息

Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, 77030, USA.

出版信息

Mod Pathol. 2020 Oct;33(10):2035-2045. doi: 10.1038/s41379-020-0564-6. Epub 2020 May 13.

Abstract

Chromosomal insertion-derived BCR-ABL1 fusion is rare and mostly cryptic in chronic myeloid leukemia (CML). Most of these cases present a normal karyotype, and their risk and/or prognostic category are uncertain. We searched our database and identified 41 CML patients (20 M/21 F, median age: 47 years, range 12-78 years) with insertion-derived BCR-ABL1 confirmed by various FISH techniques: 31 in chronic phase, 1 in accelerated phase, and 9 in blast phase at time of diagnosis. Conventional cytogenetics analysis showed a normal karyotype (n = 19); abnormal karyotype with morphologically normal chromosomes 9 and 22 (n = 5); apparent ins(9;22) (n = 2) and abnormal karyotype with apparent abnormal chromosomes 9, der(9) and/or 22, der(22) (n = 15). The locations of insertion-derived BCR-ABL1 were identified on chromosome 22 (68.3%), 9 (29.3%), and 19 (2.4%). Complex chromosomal abnormalities were often overlooked by conventional cytogenetics but identified by FISH tests in many cases. After a median follow-up of 58 months (range 1-242 months), 11 patients died, and 3 lost contact, while the others achieved different cytogenetic/molecular responses. The locations of BCR-ABL1 (der(22) vs. non-der(22)) and the karyotype results (complex karyotype vs. noncomplex karyotype) by conventional cytogenetics were not associated with overall survival in this cohort. However, redefining the complexity of chromosomal abnormality by correlating karyotype and FISH findings, CML cases with simple chromosomal abnormalities had a more favorable overall survival than that with complex chromosomal abnormalities. We conclude that insertion-derived BCR-ABL1 fusions often involve complex chromosomal abnormalities which are overlooked by conventional cytogenetics, but can be identified by one or more FISH tests. We also suggest that the traditional cytogenetic response criteria may not apply in these patients, and the complexity of chromosomal abnormalities redefined by correlating karyotype and FISH findings can plays a role in stratifying patients into more suitable risk groups for predicting prognosis. (Word count: 292).

摘要

染色体插入衍生的 BCR-ABL1 融合在慢性髓性白血病 (CML) 中较为罕见且多为隐匿性。这些病例大多表现为正常核型,其风险和/或预后分类不确定。我们检索了数据库,共确定了 41 例经各种 FISH 技术证实存在插入衍生的 BCR-ABL1 的 CML 患者(男 20 例,女 21 例,中位年龄 47 岁,范围 12-78 岁):慢性期 31 例,加速期 1 例,诊断时急变期 9 例。常规细胞遗传学分析显示核型正常(n=19);染色体 9 和 22 形态正常但核型异常(n=5);明显的 ins(9;22)(n=2)和核型异常,伴有明显的异常染色体 9、der(9)和/或 22、der(22)(n=15)。插入衍生的 BCR-ABL1 的位置分别位于染色体 22(68.3%)、9(29.3%)和 19(2.4%)。许多情况下,通过常规细胞遗传学技术往往会忽略插入衍生的 BCR-ABL1 所在的复杂染色体异常,但通过 FISH 试验可以识别。中位随访 58 个月(范围 1-242 个月)后,11 例患者死亡,3 例失访,其余患者获得不同的细胞遗传学/分子学反应。本队列中,BCR-ABL1 的位置(der(22) vs. non-der(22))和核型结果(复杂核型 vs. 非复杂核型)与总生存无相关性。然而,通过核型和 FISH 结果的相关性重新定义染色体异常的复杂性,单纯染色体异常的 CML 病例的总生存明显优于复杂染色体异常的病例。综上,插入衍生的 BCR-ABL1 融合常涉及常规细胞遗传学易忽略的复杂染色体异常,但可通过一种或多种 FISH 试验检测。我们还建议,这些患者可能不适用传统的细胞遗传学反应标准,通过核型和 FISH 结果相关性重新定义的染色体异常的复杂性可用于将患者分层为更合适的风险组,以预测预后。(词数:292)

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