6硫代鸟嘌呤治疗相关肝小静脉闭塞病六例临床分析  

Clinical characteristics of hepatic veno-occlusive disease in 6 children with hematologic neoplasm treated with 6-thioguanlne

作　　者：王宏胜[1] 高怡瑾[1] 李军[1] 陆凤娟[1] 苗慧[1] 钱晓文[1] 竺晓凡[2] 

机构地区：[1]复旦大学附属儿科医院血液科,上海201102 [2]中国医学科学院北京协和医学院血液学研究所血液病医院儿童血液病诊疗中心

出　　处：《中华儿科杂志》2010年第9期708-710,共3页Chinese Journal of Pediatrics

基　　金：基金项目：国家科技支撑计划课题资助项目（2007BA104803）

摘　　要：目的 对6例6-硫代鸟嘌呤（6-TG）治疗儿童血液肿瘤合并肝小静脉闭塞病（HVOD）患儿临床表现与转归进行初步探讨,为儿童药物相关性HVOD的防治提供参考.方法 病例来源为中国儿童白血病协作组（CCLG）部分成员单位实施CCLG急性淋巴细胞性白血病（ALL）2008方案以来2008年4月至2009年4月期间的HVOD病例6例.对6例患儿的临床表现、实验室检查及影像学、病理学及治疗进行分析.结果 6例HVOD患儿平均年龄3.89岁（3岁1个月至4岁11个月）,男2例,女4例.原发病均为ALL,多数病例有肝区疼痛症状,辅助检查可发现肝脏不同程度肿大.转氨酶及胆红素多有不同程度升高;转氨酶多可升高至正常值10倍及以上,个别病例转氨酶正常;多数胆红素升高,多数〉35μmol/L.腹水为HVOD常见症状,另外可有胸水、影像学发现肝静脉显影不清等表现.6例患儿均住院保肝、退黄及支持治疗,部分患儿予低分子肝素抗凝治疗,预后均良好,未发现死亡病例.结论 HVOD是6-TG治疗过程中的严重并发症,保肝、退黄及部分低分子肝素抗凝治疗的HVOD预后良好.Objective To improve the treatment of drug related childhood hepatic veno-occlusive disease（HVOD）,clinical characteristics of 6 children with hematologic neoplasm from 2 hospitals of China Children＇s Leukemia Group（CCLG）treated with 6-thioguanine（6-TG）complicated with HVOD were analyzed.Method All the drug related HVOD patients were treated with CCLG acute lymphoblastic leukemia（ALL）-2008 protocol They were from Children＇s Hospital of Fudan University and Institute of Hematology ＆ Blood Diseases Hospital,Chinese Academy of Medical Sciences ＆ Peking Union Medical College from April 2008 to April 2009.The diagnosis was made according to the modified Seattle criteria and Baltimore criteria,including 2 or 3 of the following clinical features：hepatomegaly and upper right abdominal pain,jaundice（bilirubin ≥ 35 μmol/L）,ascites or confirmed by pathology.The 6 HVOD patients＇ clinical manifestations,laboratory finding,imageologic and pathologic data were collected and analyzed.Result Of the 6 patients,2 were males and 4 females.Mean age of the 6 patients was 3.89years（range from 3 years 1 month to 4 years 11 months）.The original disease was acute lymphoblastic leukemia.HVOD occurred during chemotherapy protocols of CAM（CTX ＋ Ara-C ＋ 6-TG）or maintenance period（MTX ＋ 6-TG）.Most of 6 HVOD patients presented with pain in liver area,hepatomegaly on imaging,elevated aminotransferase and bilirubin（often ≥ 35 μmol/L）,hydroperitonia was common,one with pleural fluid,illegible hepatic veins.All the patients recovered after being treated with bepatoprotective,jaundice-relieving and supportive therapeutics,some patients were treated with low molecular weight heparin.The prognoses were good.Conclusion HVOD was a serious complication of chemotherapy with 6-TG.Hepatoprotective and jaundice-relieving and low molecular weight beparin could improve the prognosis.

关 键 词：硫鸟嘌呤 肝静脉闭塞性疾病 白血病 淋巴细胞 急性 儿童 

分 类 号：R575[医药卫生—消化系统]