Pramanik Raja, Tyagi Anudishi, Chopra Anita, Kumar Akash, Vishnubhatla Sreenivas, Bakhshi Sameer
Department of Medical Oncology, All India Institute of Medical Sciences, New Delhi, India.
Department of Laboratory Oncology, All India Institute of Medical Sciences, New Delhi, India.
Clin Lymphoma Myeloma Leuk. 2018 Jun;18(6):e249-e254. doi: 10.1016/j.clml.2018.03.013. Epub 2018 Mar 31.
The purpose of our study was to evaluate the clinical, cytogenetic, and molecular features, and survival outcomes in patients with acute myeloid leukemia (AML) with myeloid sarcoma (MS) and compare them with patients with AML without MS.
This was a retrospective analysis of de novo pediatric AML patients with or without MS diagnosed at our cancer center between June 2003 and June 2016.
MS was present in 121 of 570 (21.2%), the most frequent site being the orbit. Patients with MS had a younger median age (6 years vs. 10 years) and presented with higher hemoglobin and platelet but lower white blood cell count compared with patients without MS. Further, t (8; 21) (P < .01), loss of Y chromosome (P < .01), and deletion 9q (P = .03) were significantly higher in patients with AML with MS. Event-free survival (EFS; P = .003) and overall survival (OS; P = .001) were better among patients with AML with MS (median EFS 21.0 months and median OS 37.1 months) compared with those with AML without MS (median EFS 11.2 months and median OS 16.2 months). The t (8; 21) was significantly associated with MS (odds ratio, 3.92). In a comparison of the 4 groups divided according to the presence or absence of MS and t (8; 21), the subgroup of patients having MS without concomitant t (8; 21) was the only group to have a significantly better OS (hazard ratio, 0.53; 95% confidence interval, 0.34-0.82; P = .005).
Although t (8; 21) was more frequently associated with MS, it did not appear to be the reason for better outcome.
我们研究的目的是评估急性髓系白血病（AML）伴髓肉瘤（MS）患者的临床、细胞遗传学和分子特征以及生存结局，并将其与无MS的AML患者进行比较。
这是一项对2003年6月至2016年6月期间在我们癌症中心诊断的初发小儿AML伴或不伴MS患者的回顾性分析。
570例患者中有121例（21.2%）存在MS，最常见的部位是眼眶。与无MS的患者相比，MS患者的中位年龄更小（6岁对10岁），血红蛋白和血小板水平更高，但白细胞计数更低。此外，AML伴MS患者中t（8；21）（P <.01）、Y染色体缺失（P <.01）和9q缺失（P =.03）的发生率显著更高。与无MS的AML患者（中位无事件生存期11.2个月，中位总生存期16.2个月）相比，AML伴MS患者的无事件生存期（EFS；P =.003）和总生存期（OS；P =.001）更好（中位EFS 21.0个月，中位OS 37.1个月）。t（8；21）与MS显著相关（比值比，3.92）。在根据MS和t（8；21）的有无划分的4组比较中，有MS但无伴随t（8；21）的患者亚组是唯一总生存期显著更好的组（风险比，0.53；95%置信区间，0.34 - 0.82；P =.005）。
虽然t（8；21）与MS的相关性更频繁，但它似乎不是预后更好的原因。
