放线菌素D相关肝窦阻塞综合征1例及文献复习  

Actinomycin D-related sinusoidal obstruction syndrome:one case report and literature review

作　　者：路阳 董玉双 陈霁晖[1] LU Yang;DONG Yu-shuang;CHEN Ji-hui(Department of Pharmacy,School of Medicine,Shanghai Jiao Tong University,Shanghai 200092,China;Department of Pediatric Hematology/Oncology,Xinhua Hospital,School of Medicine,Shanghai Jiao Tong University,Shanghai 200092,China;Dalian Women and Children′s Medical Center Group,Dalian 116021,China)

机构地区：[1]上海交通大学医学院附属新华医院药学部,上海200092 [2]上海交通大学医学院附属新华医院儿童血液肿瘤科,上海200092 [3]大连市妇女儿童医疗中心(集团),大连116021

出　　处：《实用药物与临床》2022年第3期266-270,共5页Practical Pharmacy and Clinical Remedies

基　　金：国家自然科学基金课题(82003742)。

摘　　要：目的提高临床对放线菌素D(ACT-D)相关肝窦阻塞综合征(SOS)的认识,减少误诊、漏诊。方法回顾性分析我院诊断的1例ACT-D相关的SOS病例,并对国内外文献进行复习,分析临床特点、诊断、预防治疗对策及后续化疗方案调整。结果该患儿诊断为Ⅲ期肾母细胞瘤,首次给予ACT-D后出现发热、抽搐、肝大、腹水等临床症状,实验室检验结果显示其存在感染、黄疸、肝衰竭及血小板减少等,诊断为SOS。入院后给予积极抗感染、血浆置换等支持治疗,患儿好转并于入院第14天继续化疗,第17天出院。后续患儿继续使用全量ACT-D,未再发生SOS。结论肾母细胞瘤患儿使用ACT-D可诱发SOS,发病初期出现的原因不明的血小板减少、转氨酶升高早于总胆红素升高等情况可作为SOS发生的预警。Objective To improve the understanding of actinomycin D(ACT-D)-related hepatic sinusoidal obstruction syndrome(SOS)and reduce the incidence of misdiagnosis and missed diagnosis.Methods A retrospective analysis was performed in a patient in our hospital with ACT-D-related SOS.The relevant literature was also reviewed to analyze the clinical characteristics,diagnosis,preventive and treatment strategies,and subsequent adjustments to chemotherapy regimens.Results The patient was diagnosed with stageⅢnephroblastoma.After a single dose of ACT-D,the patient presented the clinical symptoms,including fever,convulsion,hepatomegaly and ascites.He was diagnosed as SOS according to the symptoms and the laboratory examination infection,jaundice,liver failure and thrombocytopenia.Then the patient was treated with antibiotics,plasmapheresis,and other support treatments.The patient was recovered and continued chemotherapy on day14 in hospital and was discharged on day17.The patient continued to receive full dose of ACT-D,and SOS did not recur.Conclusion SOS may occur when ACT-D is given in children with nephroblastoma.The thrombocytopenia with unknown causes and elevated transaminase earlier than the elevated total bilirubin may be warning signs of ACT-D-related SOS.

关 键 词：放线菌素D 肝窦阻塞综合征 肝小静脉闭塞病 肾母细胞瘤 

分 类 号：R969.3[医药卫生—药理学]