儿童、青少年和年轻人 (AYA) 中的费城染色体阴性骨髓增生性肿瘤 (Ph-MPN) 引起了血液学家的关注，因为由于对 Ph-MPN 的认识和诊断能力的提高，它们的发现比以前更多。发现儿童和 AYA 诊断出的 Ph-MPN 的临床特征与 60 多岁（发病高峰年龄）患者中发生的 Ph-MPN 的临床特征不同。 Ph-MPN在儿童和AYA中诊断出的三阴性病例较多，无可识别的驱动基因，血栓事件中静脉血栓形成的比例较大。在治疗方面，仍有一些年轻患者特有的问题需要解决，例如选择长期使用的细胞减灭剂以及制定妊娠和分娩期间血栓或出血事件的最佳预防措施。在本文中，我们将讨论支持这些说法的临床研究，并为日常治疗患有 Ph-MPN 的幼儿提供一些实用建议。

Philadelphia chromosome-negative myeloproliferative neoplasms (Ph- MPN) in children, adolescents, and young adults (AYA) attract attention from hematologists because they are identified more than before due to the recognition and advancement of diagnostic capacity for Ph- MPN. The clinical features of Ph- MPN diagnosed in children and AYA are found to be different from those of Ph- MPN that occur in patients in their 60s, peak age of onset. Ph- MPN diagnosed in children and AYA has more triple-negative cases with no identifiable driver genes and a larger proportion of venous thrombosis in thrombotic events. In terms of treatment, there are still problems to be resolved that are unique to younger patients, such as choosing cytoreductive agents for long-term use and the development of optimal prevention of thrombotic or bleeding events during pregnancy and childbirth. In this paper, we will discuss the clinical research supporting these claims and offer some practical advice for treating young children with Ph- MPN daily.