全反式维甲酸治疗急性早幼粒细胞白血病合并维甲酸综合征的临床研究

目的：进一步探讨全反式维甲酸（ATRA）所致维甲酸综合征（RAS）的临床特征。方法：回顾分析9例出现RAS的急性早幼粒细胞白血病的患者的临床表现、治疗与预后。结果：RAS发生时间在ATRA治疗后3～28天（中位11天）。9例发热，7例呼吸困难，4例可闻及肺部湿罗音。4例胸水，2例心包积液，5例腹水，5例下肢浮肿，3例出现尿量减少，其中1例发展成为尿毒症。9例出现至少3种上述症状。经过地塞米松治疗，减量或停用ATRA，加用化疗及对症支持治疗，RAS状均可改善。7例白血病达完全缓解，1例因颅内出血死亡。结论：RAS临床表现较为严重，需积极采用激素及相应治疗。

Clinical features of retinoic acid syndrome in acute promyelocytic leukemia treated with all-trans retinoic acid

Objective:To further explore the clinical features of all-trans retinoic acid(ATRA) caused retinoic acid syndrome(RAS).Methods:The clinical manifestations,treatment and prognosis of 9 acute promyelocytic leukemia(APL) patients with RAS were retrospectively studied.Results:Patients receiving ATRA for induction developed RAS at a median time of 11 days of ATRA (range 3-28).Fever (all of the 9 patients),respiratory distress(7/9),pulmonary infiltrates(4/9),pleural effusion(4/9),pericardial effusion(2/9),celiac effusion(5/9),crura edema(5/9),and urine decrease(3/9)were the main clinical signs.With treatment of dexamethasone,ATRA decreasing or withdraw,with or without chemotherapy,most clinic sings resolved.Seven patients got complete remission,one died of intracranial bleeding.Conclusion:RAS is a servere syndrome which need active therapy.