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目的探讨原发高白细胞型急性早幼粒细胞白血病(APL)的临床特点及有效的治疗方法。方法回顾性分析1993年10月至2006年8月苏州大学附属第一医院收治的66例原发高白细胞型APL患者和152例非高白细胞型APL患者的临床资料,并对高白细胞型患者按治疗方案的不同进行了分组比较。结果高白细胞组APL患者早期病死率、弥散性血管内凝血(DIC)和维甲酸综合征(RAS)发生率分别为30.3%,57.6%和31.8%,均高于非高白细胞组(7.2%,38.1%和21.1%)(P<0.05),而完全缓解(CR)率较低(63.6%对88.2%)(P<0.05)。高白细胞组患者中61例接受了诱导治疗,其中31例单用维甲酸治疗,21例维甲酸联合亚砷酸治疗,9例单用亚砷酸治疗,各组早期病死率分别为27.3%,14.3%和55.6%,CR率分别为67.7%,81.0%和44.4%。61例患者中41例在接受诱导分化治疗的同时加用化疗,其CR率为80.5%,总病死率为19.5%;而未加用化疗的20例患者其CR率为45.0%,总病死率为55.0%,两组比较差异有显著性意义(P<0.05)。结论原发高白细胞型APL较非高白细胞型APLCR率低,早期病死率高,DIC、RAS发生率高。维甲酸加亚砷酸双诱导并联合小剂量化疗是治疗高白细胞型APL的最有效的方案,可明显减少早期病死率,提高CR率。
Objective To investigate the clinical features and effective treatment of primary high leucocytic acute promyelocytic leukemia (APL). Methods The clinical data of 66 patients with APL with primary high white blood cell type and 152 patients with non-high leucocyte APL admitted to the First Affiliated Hospital of Soochow University from October 1993 to August 2006 were retrospectively analyzed. The clinical data of patients with high white blood cell count Different treatment options were grouped. Results The incidence of early mortality, diffuse intravascular coagulation (DIC) and retinoic acid syndrome (RAS) in high leukocyte group were 30.3%, 57.6% and 31.8%, respectively, which were significantly higher than those in non-high leukocyte group (7.2% 38.1% and 21.1% respectively) (P <0.05), while the rate of complete remission (CR) was lower (63.6% vs 88.2%) (P <0.05). Among the patients with high WBC, 61 patients received induction therapy, of which 31 were treated with retinoic acid alone, 21 were treated with retinoic acid and arsenic acid, and 9 were treated with arsenious acid alone. The early mortality rates in each group were 27.3% 14.3% and 55.6% respectively. The CR rates were 67.7%, 81.0% and 44.4% respectively. Among the 61 patients, 41 cases received chemotherapy-induced differentiation combined with chemotherapy, with a CR rate of 80.5% and a total mortality rate of 19.5%. In 20 patients without chemotherapy, the CR rate was 45.0%, and the overall mortality rate 55.0%, there was significant difference between the two groups (P <0.05). Conclusion The APL rate of primary high white blood cell type APL is lower than that of non-high white blood cell type APL, the early mortality rate is high, and the incidence of DIC and RAS is high. The combination of retinoic acid plus arsenious acid and low-dose chemotherapy is the most effective regimen for the treatment of high-leucocyte APL, which can significantly reduce the early mortality and improve the CR rate.