Pass Amy Kun, McLin Valerie A, Rushton Jennifer R, Kearney Debra L, Hastings Caroline A, Margolin Judith F
Texas Children's Cancer Center, USA.
J Pediatr Hematol Oncol. 2008 Dec;30(12):976-80. doi: 10.1097/MPH.0b013e31818b37c4.
This is the first description in which the diagnosis of vanishing bile duct syndrome (VBDS) preceded the diagnosis of Hodgkin disease (HD) by several months, and for which patients received modifications to modern MOPP-ABV chemotherapy with successful clinical remission. VBDS is an uncommon form of liver disease manifested by severe cholestasis and progressive liver failure. We report 2 cases of stage IIIB pediatric HD and VBDS. Because VBDS is progressive and the only curative treatment is liver transplant, it is imperative to recognize that children with VBDS may also have concurrent HD.
这是首次有描述表明,胆管消失综合征(VBDS)的诊断比霍奇金病(HD)的诊断早几个月,且针对这些患者对现代MOPP-ABV化疗方案进行了调整,临床缓解成功。VBDS是一种罕见的肝病形式,表现为严重胆汁淤积和进行性肝功能衰竭。我们报告2例IIIB期儿童HD合并VBDS的病例。由于VBDS是进行性的,唯一的治愈性治疗是肝移植,因此必须认识到患有VBDS的儿童可能同时患有HD。