22例分化综合征的临床分析

Clinical analysis of 22 cases of differentiation syndrome

目的探讨全反式维甲酸(ATRA)联合亚砷酸(ATO)治疗急性早幼粒细胞白血病所致的分化综合征(DS)的临床特征,复习文献归纳高危因素及防治进展。方法回顾分析我院22例分化综合征的临床表现、治疗及预后。结果本组病例中,男性多于女性,40岁以上居多,初诊白细胞计数多高于10×109/L,DS发生的时间在ATRA治疗后的3~15天,多在10天左右。临床症状中呼吸困难最常见,白细胞均较发病时升高,经地塞米松、减量或停用维甲酸、羟基脲、高三尖杉酯碱、联合化疗等治疗症状缓解。结论白细胞计数高于10×109/L、男性、年龄≥40岁、S型融合基因PML-RaRa可作为DS发生的其高危因素,及早发现DS,及早使用糖皮质激素及相应治疗可以降低死亡率。

Objective To investigate the clinical characteristics of all-trans retinoic acid （ATRA） combined with arsenic trioxid （ATO） in the treatment of acute promyelocytic leukemia-induced differentiation syndrome（DS）,and to review the high-risk factors and prevention progress. Methods The clinical manifestations,treatments and prognosis of 22 cases of differentiation syndrome were retrospectively analyzed. Results In the study,all patients included more males than females,majority more than 40 years old,were newly diagnosed with white blood cells higher than 10×10^9/L and DS occurred among 3-15 days after ATRA treatment,with average of 10 days.Dyspnea were the most common clinical manifestation,and white blood cells were higher than in the onset.But after treatment with dexamethasone,reduction or discontinuation of arsenic trioxid,hydroxyurea,homoharringtonine and combination with chemotherapy,the symptoms above achieved remission of symptoms. Conclusion White blood cell count is higher than 10×10^9/L,male,age ≥ 40 years old,Fusion Gene PML-RARA （ S ） can be considered as high-risk factors to predict DS,to discover early DS,early use of glucocorticoid and corresponding treatment can decline the mortality.