Lee Hye Ryun, Oh Bora, Hong Dae Sik, Zang Dae Young, Yoon Hwi-Joong, Kim Hyeoung Joon, Kim Inho, Ahn Jae-Sook, Cheong June-Won, Lee Kyung-A, Cho Kyung Sam, Lee Mark Hong, Bang Soo-Mee, Kim Tae Young, Yun Yeo-Min, Min Yoo Hong, Lee You Kyoung, Lee Dong Soon
Department of Laboratory Medicine, Seoul National University College of Medicine, 101 Daehakro, Jongno-gu, Seoul 110-744, Korea.
Cancer Genet Cytogenet. 2010 Dec;203(2):193-202. doi: 10.1016/j.cancergencyto.2010.08.007.
We characterized the cytogenetic changes and prognostic characteristics of 133 Korean patients with myelodysplastic syndrome (MDS), focusing on 5q- syndrome and MDS with chromosome abnormalities involving 5q deletion according to World Health Organization 2008 classification. In all patients, G banding and fluorescence in situ hybridization for 5q were performed, and in MDS patients with 5q deletion, the deleted region on chromosome 5 was mapped with fluorescence in situ hybridization for EGR1, CSF1R, and PDGFRB. The frequency of isolated del(5q) syndrome and 5q deletion was 2.2% (3 of 137 patients) and 15.3% (21 of 137 patients), respectively. International Prognostic Scoring System (IPSS) groups were low risk (5.8%), intermediate 1 (51.1%), intermediate 2 (27.8%), and high risk (15.3%). The patients with del(5q) were significantly older (62 years) and showed an unfavorable survival compared to patients without del(5q). Half (53%) of the patients with del(5q) also had complex chromosome abnormalities, including chromosome 7 abnormalities. Of the patients with del(5q), 93.3% were deleted for all three regions on 5q, compared to 66.7% of patients with isolated del(5q). Marker chromosomes proved to be chromosome 5 with interstitial deletion of q arm by fluorescence in situ hybridization in three patients. The biological characteristics of MDS in Korea seem to be markedly different from those of Caucasians, with Koreans having a younger age, lower frequencies of 5q- syndrome, higher frequencies of complex cytogenetic abnormalities including del(5q), and poorer prognosis. We infer that additional chromosome abnormalities contribute to the adverse prognostic impact in patients with del(5q).
我们对133例韩国骨髓增生异常综合征(MDS)患者的细胞遗传学变化和预后特征进行了研究,重点关注根据世界卫生组织2008年分类的5q-综合征以及伴有涉及5q缺失的染色体异常的MDS。对所有患者进行了G显带和5q的荧光原位杂交检测,对于伴有5q缺失的MDS患者,利用针对EGR1、CSF1R和PDGFRB的荧光原位杂交技术对5号染色体上的缺失区域进行定位。孤立性del(5q)综合征和5q缺失的发生率分别为2.2%(137例患者中的3例)和15.3%(137例患者中的21例)。国际预后评分系统(IPSS)分组为低危(5.8%)、中危1(51.1%)、中危2(27.8%)和高危(15.3%)。与无del(5q)的患者相比,del(5q)患者年龄显著更大(62岁),生存情况不佳。del(5q)患者中有一半(53%)还存在复杂的染色体异常,包括7号染色体异常。在del(5q)患者中,93.3%的患者5号染色体上的所有三个区域均缺失,而孤立性del(5q)患者的这一比例为66.7%。通过荧光原位杂交证实,3例患者的标记染色体为5号染色体,q臂存在间质性缺失。韩国MDS的生物学特征似乎与白种人明显不同,韩国患者年龄更小,5q-综合征发生率更低,包括del(5q)在内的复杂细胞遗传学异常发生率更高,预后更差。我们推断,额外的染色体异常对del(5q)患者的不良预后有影响。