目的：：分析儿童肿瘤患者化疗相关肝静脉阻塞综合征(Hepatic veno-occlusive disease,HVOD)的临床特征和预后情况,提高临床诊治水平。方法：回顾性总结我院近3年诊治的5例化疗相关 HVOD患儿的临床特征及诊治经过。结果：基础疾病包括急性淋巴细胞白血病4例和腺泡型横纹肌肉瘤1例。年龄2岁10月至14岁,男性1例,女性4例。ALL患者并发 HVOD均发生于CAT巩固化疗阶段,腺泡型横纹肌肉瘤患儿 HVOD于 IVA方案后第5天起病。5例患儿临床上均存在显著肝脏肿大、腹水、体重增加、高胆红素血症和肝酶升高,符合Baltimore诊断标准。血小板显著降低和输注无效为本组 HVOD重要临床特征。此外,患者存在严重骨髓抑制,胸部CT检查证实2例患儿存在明确的肺部感染。腹部多普勒超声波检查发现2例患者门静脉血流异常。经积极对症支持治疗和前列腺素 E1输注,5例患儿均病情恢复,无死亡病例。结论：HVOD为肿瘤患儿化疗相关严重并发症,6-硫鸟嘌呤(6-TG)为化疗相关 HOVD常见化疗药物。注重识别 HVOD临床表现,动态监测肝功、出凝血和腹部超声波检查,依据病情严重程度尽早给予综合性治疗措施,有助于避免病情进展,降低死亡率。%Objective:To review the clinical features of chemotherapy-associated hepatic veno-occlusive disease (HVOD),in order to improve the clinical diagnosis and management of HVOD.Methods:The clinical data of 5 children with chemotherapy-associated HVOD diagnosed in our hospital from September 2013 to January 2015 was analyzed retrospectively.Results:Patients enrolled in this study included 4 children with acute lymphoblastic leukemia (ALL)and 1 child with alveolar rhabdomyosarcoma (aRMS),including 4 girls and 1 boy,aged from 2 year and 10 months to 14 years.HVOD occurred during CAT (cyclophosphamide+cytosine arabino-side +6-thioguanine)consolidation chemotherapy in 4 children with ALL,and 5 days after IVA (Ifosfamide+Vincristine+Actino-mycin )induction therapy in the patient with aRMS.Clinically,the 5 cases complicated with chemotherapy-associated HVOD were all characterized by hepatomegaly with right upper quadrant pain,ascites,weight gain,hyperbilirubinemia and elevated liver en-zymes,consistent with the Baltimore diagnostic criteria of HVOD.In addition,the patients presented with profound thrombocytope-nia at the onset of HVOD,which were refractory to repeated platelet transfusions.Myosuppression was also quite severe and pneu-monia was documented by chest CT scanning in 2 cases.Of note,abnormal or reverted blood flows in the portal veins were disclosed by abdominal Doppler ultrasonography.With patient-tailored symptomatic and supportive therapeutic measures,including prosta-glandin E1,all 5 patients recovered.Conclusion:HVOD is sometimes complicated during chemotherapy,and 6-TG is associated with an increased risk of HOVD.Close observation of common clinical manifestation of HOVD,dynamic laboratory investigations of liver function and coagulation,and imaging studies are important in the early diagnosis and prompt management of HVOD,which aids in preventing disease progression and mortality.
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