Mansoor Neelum, Jabbar Naeem, Arshad Uzma, Maqsood Sidra, Habib Muhammad A, Raza Muhammad R
The Indus Hospital.
Jinnah Medical College Hospital, Karachi, Pakistan.
J Pediatr Hematol Oncol. 2020 Aug;42(6):e423-e427. doi: 10.1097/MPH.0000000000001853.
Childhood acute myeloid leukemia (AML) harboring core binding factor (CBF)-associated translocations are considered as a favorable cytogenetic subgroup. The 2 major subtypes of CBF-AML include t(8;21) and inversion of chromosome 16, accounting for ∼25% of patients. Because of expensive and toxic treatment, which may require hospitalization during the entire course of induction chemotherapy, most of the centers in Pakistan neither workup for this low-risk entity nor offer curative treatment. Therefore, we adopted an approach of screening AML cases for the presence of CBF with the rationale of offering curative treatment to this subgroup. Data of 244 cases were reviewed, and translocations were found in 72 (34%) patients among them, 59 (82%) had t(8;21) and 13 (18%) showed inversion of chromosome 16. The event-free survival with and without abandonment was 36% and 40%, respectively. Among 44 patients who completed treatment, 26 (59%) are leukemia-free, while 18 (41%) relapsed. None of the relapsed patients received salvage chemotherapy or hematopoietic stem cell transplant. Treatment-related mortality and abandonment was found in 24% and 10% of patients, respectively. The frequency of CBF-AML is higher in our study; however, poor outcome demands holistic measures in supportive care to improve the survival.
伴有核心结合因子（CBF）相关易位的儿童急性髓系白血病（AML）被认为是一个预后良好的细胞遗传学亚组。CBF-AML的2种主要亚型包括t(8;21)和16号染色体倒位，占患者的25%左右。由于治疗费用高昂且有毒性，在诱导化疗的整个过程中可能需要住院治疗，巴基斯坦的大多数中心既不对这种低风险类型进行检查，也不提供治愈性治疗。因此，我们采用了一种筛查AML病例中CBF存在情况的方法，目的是为该亚组提供治愈性治疗。回顾了244例病例的数据，其中72例（34%）患者发现有易位，59例（82%）有t(8;21)，13例（18%）显示16号染色体倒位。有放弃治疗和无放弃治疗的无事件生存率分别为36%和40%。在44例完成治疗的患者中，26例（59%）无白血病，18例（41%）复发。复发患者均未接受挽救性化疗或造血干细胞移植。治疗相关死亡率和放弃治疗率分别在24%和10%的患者中出现。在我们的研究中，CBF-AML的发生率较高；然而，不良预后需要在支持治疗中采取整体措施以提高生存率。
