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黎巴嫩一家主要转诊中心成人急性髓系和淋巴细胞白血病的分子特征分析:一份10年经验报告及文献综述

Molecular profiling of adult acute myeloid and lymphoid leukemia in a major referral center in Lebanon: a 10-year experience report and review of the literature.

作者信息

Assaf Nada, El-Cheikh Jean, Bazarbachi Ali, Salem Ziad, Farra Chantal, Chakhachiro Zaher, Nassif Samer, Zaatari Ghazi, Mahfouz Rami

机构信息

Molecular Diagnostics Laboratory, Department of Pathology and Laboratory Medicine, American University of Beirut Medical Center, PO BOX 11-0236, Beirut, Lebanon.

Department of Internal Medicine, Basile Cancer Center, American University of Beirut Medical Center, Beirut, Lebanon.

出版信息

Mol Biol Rep. 2019 Apr;46(2):2003-2011. doi: 10.1007/s11033-019-04649-2. Epub 2019 Jan 30.

Abstract

Recurrent genetic abnormalities confer distinct morphologic features and play a role in determining the clinical behavior, prognosis and adequate treatment of acute leukemia. In the MENA region, only one study targets the frequency of genetic modifications in AML, reporting a higher occurrence of acute promyelocytic leukemia in Lebanon. Determining the frequency of translocations and gene mutations in acute myeloid and lymphoid leukemia cases in an adult patients' population in Lebanon and comparing the resultant genetic profile with the published international molecular profile of adult acute leukemia. Laboratory results of adult patients diagnosed with AML or ALL presenting to AUBMC for genetic profiling between years 2006 until June 2016 were reviewed. Genetic profiling of AML cases in our CAP accredited molecular diagnostics laboratory consists of a validated lab developed RT-PCR for the detection of RUNX1/RUNX1T1, CBFB/MYH11, KMT2A/MLLT3, PML-RARA, and BCR-ABL and mutations in the FLT3 receptor, NPM1, c-kit and CEPBA genes. The ALL panel tests for the presence of BCR-ABL1, ETV6/RUNX1; KMT2A/AFF1, and TCF3-PBX1. We reviewed 580 AML and 175 ALL cases. In the AML cohort, the M:F ratio was 1.3:1 with a mean age of 50 years. t(15;17) was present in 7.6%, t(8;21) in 4.2%, inv(16) in 3.7%, t(9;22) in 2.2% and t(9;11) in 1.7% of cases. FLT3 mutation (ITD or TKD) was present in 25.2% of all cases and 30.1% of Cytogenetics-normal (CN) patients. Mutations of the NPM1 gene was present in 31.4% of AML cases and in 43.8% of CN patients. Double positive (NPM1+/FLT3+) cases accounted for 20% of NK patients. CEBPA and c-kit mutations were detected in 7.3% and 2.4% respectively. In the ALL cohort, the mean age was 37 years. B- and T-lymphoblastic leukemia constituted 84.6% and 15.4% of ALL cases and the M:F ratio was 1.2:1 and 2.86:1 respectively. B-ALL patients were positive for t(9;22) in 14.2%, t(4;11) in 5.4%, t(1;19) in 2.7% and t(12;21) in 1.4%. T-ALL patients were negative for translocations found in our ALL panel. A lower mean age was found in our adult leukemic Lebanese population as compared to the Western cases. Other interesting findings were the lower percentage of inv(16), lower incidence of TCF3-PBX1, and the mild increase in Philadelphia positivity in our AML cohort. In our ALL cohort, t(9;22) positivity was less than expected for adult lymphoblastic leukemia. Full molecular profiling by next generation sequencing is required for further classification of cases into prognostic categories. This study will be a baseline reference for future research and epidemiological data useful for transplant centers and oncologists both in Lebanon and the region.

摘要

复发性基因异常赋予独特的形态学特征,并在决定急性白血病的临床行为、预后及适当治疗方面发挥作用。在中东和北非地区,仅有一项研究针对急性髓系白血病(AML)的基因改变频率,报告称黎巴嫩急性早幼粒细胞白血病的发病率较高。本研究旨在确定黎巴嫩成年患者群体中急性髓系和淋巴细胞白血病病例的易位和基因突变频率,并将所得基因图谱与已发表的成人急性白血病国际分子图谱进行比较。回顾了2006年至2016年6月期间在贝鲁特美国大学医疗中心(AUBMC)进行基因分析的成年AML或ALL患者的实验室结果。我们经美国病理学家协会(CAP)认可的分子诊断实验室对AML病例的基因分析包括用于检测RUNX1/RUNX1T1、CBFB/MYH11、KMT2A/MLLT3、PML-RARA和BCR-ABL以及FLT3受体、NPM1、c-kit和CEBPA基因突变的经过验证的实验室自行研发的逆转录聚合酶链反应(RT-PCR)。ALL检测项目用于检测BCR-ABL1、ETV6/RUNX1、KMT2A/AFF1和TCF3-PBX1的存在情况。我们回顾了580例AML和175例ALL病例。在AML队列中,男女比例为1.3:1,平均年龄为50岁。15;17)易位在7.6%的病例中出现,8;21)易位在4.2%的病例中出现,16号染色体倒位(inv(16))在3.7%的病例中出现,9;22)易位在2.2%的病例中出现,9;11)易位在1.7%的病例中出现。FLT3突变(内部串联重复突变[ITD]或酪氨酸激酶结构域突变[TKD])在所有病例的25.2%以及细胞遗传学正常(CN)患者的30.1%中出现。NPM1基因突变在31.4%的AML病例以及CN患者的43.8%中出现。双阳性(NPM1+/FLT3+)病例占CN患者的20%。CEBPA和c-kit突变分别在7.3%和2.4%的病例中检测到。在ALL队列中,平均年龄为37岁。B淋巴细胞白血病和T淋巴细胞白血病分别占ALL病例的84.6%和15.4%,男女比例分别为1.2:1和2.86:1。B-ALL患者中14.2%存在9;22)易位,5.4%存在4;11)易位,2.7%存在1;19)易位,1.4%存在12;21)易位。T-ALL患者在我们的ALL检测项目中未发现易位。与西方病例相比,我们黎巴嫩成年白血病患者群体的平均年龄较低。其他有趣的发现包括我们AML队列中16号染色体倒位的比例较低、TCF3-PBX1的发病率较低以及费城染色体阳性率略有增加。在我们的ALL队列中,9;22)易位阳性率低于成人淋巴细胞白血病的预期值。需要通过下一代测序进行全面的分子分析,以便将病例进一步分类为预后类别。本研究将成为未来研究的基线参考以及对黎巴嫩和该地区的移植中心及肿瘤学家有用的流行病学数据。

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