Department of Pediatrics, Division of Hematology/Oncology, Weill Cornell Medicine, 525 E. 68th St., Payson-695, New York, NY, 10065, USA.
Curr Hematol Malig Rep. 2020 Apr;15(2):141-148. doi: 10.1007/s11899-020-00571-8.
Myeloproliferative neoplasms are traditionally seen in older adults, making them poorly understood in younger patients. Clinical presentation, genetic landscape, outcomes, and best management practices are inadequately described in this group. Over the past decade, more research has focused on younger patients, and this paper seeks to review and describe the current status of the field.
A recent review analyzed the available pediatric MPN literature and highlighted the paucity of published data. Pediatric patients showed lower rates of the common mutations found in adults, thrombotic events, and disease transformation to myelofibrosis and acute leukemia. A number of centers have recently shared their experience with young adult patients. Better survival outcomes were confirmed for young adult patients compared to older patients. There is still much to learn about myeloproliferative neoplasms in pediatric and young adult patients, but currently available data showing better outcomes is reassuring.
骨髓增殖性肿瘤传统上见于老年人,因此在年轻人中了解甚少。在这一人群中,临床表现、遗传特征、预后和最佳治疗方法都描述不足。在过去十年中,更多的研究集中在年轻患者,本文旨在回顾和描述该领域的现状。
最近的一项综述分析了现有的儿科骨髓增殖性肿瘤文献,并强调了发表数据的缺乏。儿科患者常见的突变率低于成年人,血栓事件和疾病向骨髓纤维化和急性白血病转化的发生率也较低。最近有许多中心分享了他们在年轻成年患者中的经验。与老年患者相比,年轻成年患者的生存结果更好。关于儿科和年轻成年患者的骨髓增殖性肿瘤,我们还有很多需要了解,但目前显示更好结果的可用数据令人欣慰。