DeBenedet Anthony T, Berg Carl L, Enfield Kyle B, Woodford Randall L, Bennett Audrey K, Northup Patrick G
Department of Internal Medicine, University of Michigan Health System, Ann Arbor, Michigan 48109-0368, USA.
Nat Clin Pract Gastroenterol Hepatol. 2008 Jan;5(1):49-53. doi: 10.1038/ncpgasthep1001.
A 39-year-old man presented with a 2-month history of abdominal pain, jaundice, non-bloody diarrhea, weakness, and weight loss. Initial evaluation revealed intrahepatic ductopenia consistent with vanishing bile duct syndrome and IBD, type unclassified. Although treatment with budesonide improved his symptoms, they worsened several months later. On repeat evaluation, he was found to have extensive lymphadenopathy and an elevated white blood cell count.
Physical examination, laboratory investigations, abdominal ultrasound, CT scans, magnetic resonance cholangiopancreatography, endoscopic retrograde cholangiopancreatography, colonoscopies with biopsies, hepatic biopsy, axillary lymph node biopsy.
Hodgkin's lymphoma with secondary vanishing bile duct syndrome and IBD, type unclassified.
The initial symptoms were managed with budesonide, but following recurrence, the patient's underlying lymphoma was treated with nitrogen mustard and dexamethasone.
一名39岁男性，有2个月的腹痛、黄疸、非血性腹泻、乏力及体重减轻病史。初步评估显示肝内胆管减少，符合胆管消失综合征及未分类的炎症性肠病。尽管使用布地奈德治疗后症状有所改善，但数月后病情恶化。再次评估时，发现他有广泛的淋巴结病且白细胞计数升高。
体格检查、实验室检查、腹部超声、CT扫描、磁共振胰胆管造影、内镜逆行胰胆管造影、结肠镜检查及活检、肝活检、腋窝淋巴结活检。
霍奇金淋巴瘤伴继发性胆管消失综合征及未分类的炎症性肠病。
最初症状用布地奈德治疗，但复发后，患者的潜在淋巴瘤用氮芥和地塞米松治疗。
