Department of Hematology, G. Papanikolaou Hospital of Thessaloniki, Exochi 57010, Greece.
Mediterr J Hematol Infect Dis. 2011;3(1):e2011038. doi: 10.4084/MJHID.2011.038. Epub 2011 Sep 8.
Acute promyelocytic leukemia (APL) with WBC above 10 G/L has long been considered, even in the all-trans retinoic acid (ATRA) era, to carry a relatively poor prognosis (compared to APL with WBC below 10 G/L), due to increased early mortality and relapse. However, early deaths can to a large extent be avoided if specific measures are rapidly instigated, including prompt referral to a specialized center, immediate onset of ATRA and chemotherapy, treatment of coagulopathy with adequate platelet transfusional support, and prevention and management of differentiation syndrome. Strategies to reduce relapse rate include chemotherapy reinforcement with cytarabine and/or arsenic trioxide during consolidation, prolonged maintenance treatment, especially with ATRA and low dose chemotherapy, and possibly, although this is debated, intrathecal prophylaxis to prevent central nervous system relapse. By applying those measures, outcomes of patients with high risk APL have considerably improved, and have become in many studies almost similar to those of standard risk APL patients.
高白细胞急性早幼粒细胞白血病（APL）（白细胞计数＞10×109/L）在全反式维甲酸（ATRA）时代，即使与白细胞计数＜10×109/L 的 APL 相比，也被认为预后相对较差，这主要是因为早期死亡率和复发率增加。然而，如果迅速采取特定措施，包括迅速转至专门中心、立即开始 ATRA 和化疗、用足够的血小板输注治疗凝血障碍，以及预防和管理分化综合征，就可以在很大程度上避免早期死亡。降低复发率的策略包括在巩固治疗期间用阿糖胞苷和/或三氧化二砷强化化疗、延长维持治疗，特别是用 ATRA 和低剂量化疗，以及可能（虽然存在争议）预防性鞘内化疗以预防中枢神经系统复发。通过应用这些措施，高危 APL 患者的结局得到了显著改善，在许多研究中几乎与标准风险 APL 患者相似。
