Division of Oncology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.
Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, USA.
Pediatr Blood Cancer. 2023 Aug;70(8):e30401. doi: 10.1002/pbc.30401. Epub 2023 May 9.
There are limited data pertaining to the prognostic features and optimal therapeutic approach for the 20%-25% of children with lymphoblastic lymphoma (LLy) who have the B-lymphoblastic subtype. Outcomes are favorable following treatment modeled after acute lymphoblastic leukemia (ALL) regimens, but prognosis is dismal after relapse, and there are no established features for predicting therapy response. Ongoing US and international trials will include the largest cohort of uniformly treated patients with B-LLy to date, providing an opportunity to define clinical and molecular predictors of relapse and to establish a standard of care for treatment to improve outcomes for this rare pediatric cancer.
对于 20%-25%患有淋巴母细胞淋巴瘤（LLy）的儿童，他们的 B 淋巴母细胞亚型预后特征和最佳治疗方法的数据有限。按照急性淋巴细胞白血病（ALL）方案治疗后，结果是有利的，但复发后预后不佳，而且目前还没有确定的特征可以预测治疗反应。目前正在进行的美国和国际试验将包括迄今为止最大的一组统一治疗的 B-LLy 患者队列，这为确定复发的临床和分子预测因素以及为改善这种罕见儿科癌症的治疗效果建立护理标准提供了机会。
