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

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.