霍奇金病伴胆管减少（消失性胆管综合征）的胆汁淤积性肝病：病例报告

Cholestatic liver disease with ductopenia (vanishing bile duct syndrome) in Hodgkin's disease: report of a case.

作者信息

Guliter Sefa, Erdem Ozlem, Isik Mine, Yamac Kadri, Uluoglu Omer

机构信息

University of Kirikkale, Faculty of Medicine, Department of Gastroenterology, Kirikkale, Turkey.

出版信息

Tumori. 2004 Sep-Oct;90(5):517-20. doi: 10.1177/030089160409000516.

DOI:10.1177/030089160409000516

PMID:15656342

Abstract

Liver involvement is common in advanced stages of Hodgkin's disease. However, only a small percentage of patients with Hodgkin's disease develops jaundice due to several causes. Vanishing bile duct syndrome secondary to Hodgkin's disease is a rare cause of cholestasis in these patients. Only 20 cases, to our knowledge, have been reported so far in adults. We report a case of Hodgkin's disease presenting with obstructive jaundice without detectable liver involvement. Liver biopsies revealed intrahepatic cholestasis and ductopenia. Although the patient was given chemotherapy, he died of sepsis and disseminated intravascular coagulation after 24 weeks of admission to hospital.

摘要

肝脏受累在霍奇金淋巴瘤晚期很常见。然而，由于多种原因，只有一小部分霍奇金淋巴瘤患者会出现黄疸。霍奇金淋巴瘤继发的消失性胆管综合征是这些患者胆汁淤积的罕见原因。据我们所知，迄今为止成人中仅报告了20例。我们报告1例表现为梗阻性黄疸但无肝脏受累迹象的霍奇金淋巴瘤病例。肝脏活检显示肝内胆汁淤积和胆管减少。尽管对该患者进行了化疗，但他在入院24周后死于败血症和弥散性血管内凝血。