复杂核型急性早幼粒细胞白血病治疗过程中并发维甲酸综合征一例  

Acute promyelocytic leukemia with complex karyotype complicated by retinoic acid syndrome during treatment:one case report

作　　者：寇明坤 吴涛[1] 汉英[1] 薛锋[1] 毛东锋 潘耀柱[1] 王存邦[1] 白海[1] Kou Mingkun;Wu Tao;Han Ying;Xue Feng;Mao Dongfeng;Pan Yaozhu;Wang Cunbang;Bai Hai(Hematological Disease Center,Lanzhou General Hospital,Lanzhou Command,Lanzhou 730050,Gansu Province,China)

机构地区：[1]兰州军区兰州总医院全军血液病中心,730050

出　　处：《国际输血及血液学杂志》2020年第1期57-61,共5页International Journal of Blood Transfusion and Hematology

摘　　要：目的探讨急性早幼粒细胞白血病(APL)治疗过程中并发维甲酸综合征(RAS)的治疗方法。方法选择2018年12月28日,兰州军区兰州总医院全军血液病中心收治的1例治疗过程中并发RAS的复杂核型APL患者为研究对象。采用回顾性分析方法,收集本例患者的临床病例资料,并对其临床表现和诊治过程进行分析。本例患者的APL治疗方案为全反式维甲酸(ATRA)+三氧化二砷(ATO)诱导化疗:ATRA 20 mg/次,2次/d,口服,d1~28;ATO 10mL/d,静脉注射,d1~14;RAS治疗方案为减量或停用ATRA或ATO,尽早静脉注射地塞米松10 mg/次,2次/d,直至RAS相关临床症状明显改善。当患者白细胞计数(WBC)>10×109/L且持续升高时,酌情加用蒽环类药物或阿糖胞苷。本研究遵循的程序符合2013年修订版《世界医学协会赫尔辛基宣言》要求,并且与受试者签署临床研究知情同意书。结果本例患者入院完善相关实验室及辅助检查后,于2019年1月1日确诊为APL,PML-RARα(Bcr1型)阳性、复杂核型、中危组。患者接受ATRA+ATO方案诱导化疗后,疗效良好。患者接受ATRA治疗后,出现发热、呼吸衰竭、胸腔积液、WBC升高等临床表现,考虑其发生RAS。患者经地塞米松、吡柔比星及对症治疗后,其RAS相关临床症状明显改善。截至2019年2月,患者一般状况良好,目前仍在定期随访中。结论ATRA+ATO方案对APL患者疗效良好。APL患者治疗过程中发生RAS时,应积极采用激素及对症治疗。由于本文仅对1例患者进行回顾性研究,本研究对该病采用的治疗方案的确切疗效,尚需扩大样本量进一步验证。Objective To explore the treatment of acute promyelocytic leukemia(APL),and the management of retinoic acid syndrome(RAS)during treatment of APL.Methods On December 28,2018,one case of APL patient with complex karyotype complicated by RAS who was admitted to the Hematological Disease Center of Lanzhou General Hospital,was selected as the subject.By retrospective analysis,the clinical data of this patient were collected,and the clinical manifestations,diagnosis and treatment process were analyzed.Induction chemotherapy of all-trans retinoic acid(ATRA)combined with arsenic trioxide(ATO)was used to treat APL:ATRA 20 mg/time,twice a day,oral,d1-28;ATO 10 mL/d,intravenous injection,d1-14.The RAS treatment regimen was to reduce or discontinue ATRA or ATO,and intravenous injected dexamethasone 10 mg/time,twice a day as soon as possible until hypoxemia is relieved.When patient′s white blood cell count(WBC)>10×109/L and continuously elevated,anthracycline or cytarabine was administrated as appropriate.The procedure followed in this study were in accordance with the requirements of the World Medical Association Declaration of Helsinki revised in 2013.And this patient signed the informed consents for clinical trials.Results On January 1,2019,the patient was diagnosed as APL,with PML-RARα(Bcr1 type)positive,complex karyotype and intermediate risk group,based on complete results of relevant laboratory and auxiliary examination.This patient achieved good efficacy after treatment of ATRA+ATO regimen.After treatment with ATRA,the patient presented fever,respiratory failure,pleural effusion,and increased WBC,etc..Then RAS was considered.After treatment with dexamethasone,pirarubicin and symptomatic treatment,the patient′s clinical symptoms of RAS were significantly improved.As of February 2019,the patient was generally in good condition and was currently being followed up regularly.Conclusions ATRA+ATO regimen has a good efficacy in treatment of APL.When RAS appears during the treatment,glucocorticoids and corresponding tre

关 键 词：白血病 早幼粒细胞 急性 维甲酸 砷剂 维甲酸综合征 PML-RARΑ融合基因 全反式维甲酸 三氧化二砷 地塞米松 回顾性研究 

分 类 号：R733[医药卫生—肿瘤]