摘要目的：探讨急性早幼粒细胞白血病（APL）患者中高白细胞的发生率、临床特征及早期疗效。方法回顾性分析初诊APL患者248例，其中高白细胞患者70例，分析高白细胞的发生率、临床特征及诱导治疗的疗效。非高白细胞APL的诱导治疗以全反式维甲酸联合三氧化二砷为主，高白细胞APL的诱导治疗以全反式维甲酸+三氧化二砷+蒽环类化疗药物为主，同时给予对症支持治疗。结果248例患者中，高白细胞的发生率为28.2%（70／248），其中WBC为10×109／L～50×109／L 46例（18.5%）， WBC＞50×109／L且≤100×109／L 14例（5.6%），WBC＞100×109／L 10例（4.0%）。高白细胞APL患者的临床特征为PML-RARα融合基因S型（P＝0.004）及FLT3-ITD基因突变（P＜0.01），原发性纤溶亢进更加严重；诱导治疗完全缓解率低于非高白细胞APL患者[88.9%（48／54）比98.1%（152／155），P＝0.013]，早期死亡率高于非高白细胞患者[11.1%（6／54）比1.9%（3／155），P＝0.013]。高白细胞患者的早期死亡原因主要为脑出血。结论高白细胞是APL不良预后因素，诱导治疗应重视凝血异常的纠正、联合化疗迅速降低白细胞，以及联合地塞米松预防诱导分化综合征，以期降低早期死亡率。

abstractsObjective To investigate the rate, clinical characteristics and therapeutic efficacy of the newly diagnosed acute promyelocytic leukemia (APL) with high WBC. Methods 248 newly diagnosed APL patients were analyzed retrospectively, including 70 patients with high WBC, and the incidence, clinical characteristics and therapeutic efficacy were investigated. The patients with WBC≤10 ×109/L mainly used ATRA plus As2O3, and the patients with WBC>10 ×109/L mainly used ATRA, As2O3 plus chemotherapy (anthracycline alone or with cytarabine), the supportive care was given at the same time. Results Of the 248 patients, the rate of high WBC was 28.2%, including 18.5%patients with initial WBC (10~50)×109/L, 5.6%patients with WBC (50~100) ×109/L and 4 % patients with WBC>100 ×109/L. The clinical characteristics of patients with high WBC were the short PML-RARαisoform (P=0.004), FLT3-ITD mutations (P<0.01), and the more serious primary hyperfibrinolysis. The complete response (CR) rate in patients with high WBC was lower than that in the patients without high WBC [88.9 % (48/54) vs. 98.1 % (152/155), P=0.013], but the early death (ED) rate was higher [11.1 % (6/54) vs. 1.9 % (3/155), P=0.013). The main cause of ED in patients with WBC>10 ×109/L was cerebral hemorrhage. Conclusions The high WBC is a poor prognostic factor for the APL.During the induction therapy, it should be paid more attention to correct the coagulopathy, combine with chemotherapy to reduce the WBC rapidly, as well as use dexamethasone to prevent the differentiation syndrome, in order to reduce the ED rate.