Department of Laboratory Medicine, Chungbuk National University Hospital, Cheongju-si, Korea.
Department of Laboratory Medicine, Chungbuk National University College of Medicine, Cheongju-si, Korea.
J Clin Lab Anal. 2023 Feb;37(3):e24839. doi: 10.1002/jcla.24839. Epub 2023 Jan 19.
Recently, multigene target sequencing is widely performed for the purpose of prognostic prediction and application of targeted therapy. Here, we proposed a new scoring system that encompasses gene variations, telomere length, and Revised International Prognostic Scoring System (IPSS-R) together in Asian myelodysplastic syndrome.
We developed a new scoring model of these variables: age ≥ 65 years + IPSS-R score + ASXL1 mutation + TP53 mutation + Telomere length (<5.37). According to this new scoring system, patients were divided into four groups: very good score cutoff (≤3.0), good (3.0-4.5), poor (4.5-7.0), and very poor (>7.0).
The median OS was 170.1, 100.4, 46.0, and 12.0 months for very good, good, poor, and very poor, retrospectively (p < 0.001). Meanwhile, according to the conventional IPSS-R scoring system, the median OS was 141.3, 50.2, 93.0, 36.0, and 16.2 months for very low, low, intermediate, high, and very high, retrospectively (p < 0.001).
The newly developed model incorporating molecular variations and TL yielded more clear separations of the survival curves. By adding the presence of gene mutation and telomere length to the existing IPSS-R, its predictive ability can be further improved in myelodysplastic syndrome.
最近，多基因靶向测序被广泛应用于预后预测和靶向治疗。在这里，我们提出了一种新的评分系统，该系统将基因变异、端粒长度和修订后的国际预后评分系统（IPSS-R）结合在一起，用于亚洲骨髓增生异常综合征。
我们建立了一个新的评分模型，包含这些变量：年龄≥65 岁+IPSS-R 评分+ASXL1 突变+TP53 突变+端粒长度（<5.37）。根据这一新的评分系统，患者被分为四组：非常好的评分截定点（≤3.0）、好（3.0-4.5）、差（4.5-7.0）和非常差（>7.0）。
回顾性分析显示，非常好、好、差和非常差组的中位 OS 分别为 170.1、100.4、46.0 和 12.0 个月（p<0.001）。同时，根据传统的 IPSS-R 评分系统，非常低、低、中、高和非常高组的中位 OS 分别为 141.3、50.2、93.0、36.0 和 16.2 个月（p<0.001）。
新的模型纳入了分子变异和 TL，使生存曲线的分离更加清晰。通过在现有的 IPSS-R 中加入基因突变和端粒长度的存在，可以进一步提高骨髓增生异常综合征的预测能力。
