急性早幼粒细胞白血病分化综合征临床特征及对预后的影响研究

Differentiation syndrome in patients with acute promyelocytic leukemia:clinical features and prognosis

目的探讨急性早幼粒细胞白血病(APL)分化综合征(DS)的临床特征和影响预后的因素。方法收集中山大学附属第一医院2003—2010年收治的97例APL患者,采用维甲酸或维甲酸联合三氧化二砷(ATO)双诱导治疗,初诊者诱导分化治疗后WBC≥5×109/L联合化疗,完全缓解后采用ATO联合常规化疗巩固治疗方案。结果27例(27.83%)并发DS,出现DS中位时间为诱导分化治疗第6(2～24)天,其中24例(88.89%)发生在第1、2周。27例DS均给予地塞米松治疗,其中12例暂时停用诱导分化剂患者DS症状均缓解,15例继续使用者2例(7.41%)病情恶化死亡。中位随访37个月,DS组与未并发DS组患者无病生存(LFS)差异无统计学意义(P=0.269)。分析与DS发生的相关因素表明DS更多见于初诊WBC>10×109/L患者(χ2=4.994)。结论初诊WBC>10×109/L患者易并发DS,DS主要发生在诱导分化治疗前2周,DS对长期预后无影响。

Objective To study the clinical features and prognosis of differentiation syndrome （DS） in acute promyeloeytic leukemia （APL）. Methods We enrolled 97 patients with APL who received induction treatment with all-trans retinoic acid （ATRA） alone or in combination with arsenic trioxide （ATO） in the First Affiliated Hospital of Sun Yat-sen University from 2003 to 2010. Newly-diagnosed patients and those with WBC count ≥5 × 10^9/L after differentiation induction received combined ATRA ＋ ATO chemotherapy followed by combined ATO and conventional chemotherapy regimen for maintenance after complete remission. Results During induction differentiation therapy ,27 （27.83 % ） patients developed DS at a median of 6 （range ：2- 24） days, of whom 24 （88.89%） in the initial two weeks. Of 27 patients with DS given dexamethasone treatment ,symptoms resolved in 12 patients who temporarily discontinued differentiation medication. Of 15 patients who continued differentiation therapy ,2（7.41% ） died due to deterioration of DS. The median follow-up duration was 37 months. The difference in leukemia-free survival was unremarkable in those with or without DS （P = 0. 269 ）. Patients with WBC 〉 10 × 10^9/L on initial diagnosis were more inclined to develop DS （X2 = 4. 994） , as was indicated by DS-associated factors. Conclusion Patients with WBC 〉 10 × 10^9/L on initial diagnosis are at higher risk of developing DS ,which mainly occurs in the initial two weeks of induction therapy. No impact of DS on long-term leukemia-free survival is identified.