opsy revealed marked cellular and canalicular perivenular We report the case of a 21-year-old woman who developed cholestasis. Bile ducts were absent in all five portal tracts severe adult onset ductopenia in association with Hodgkin's present, two of which had easily recognizable hepatic arterilymphoma. Chemotherapy resulted in a remission of her ole branches. The patient was started on prednisolone 60 Hodgkin's disease (HD) and significant improvement in liver mg/d and discharged. function with resolution of the hepatic and biliary duct histo-Two weeks later, the patient was readmitted with deepenlogical abnormalities, a therapeutic success not previously ing jaundice, a fluctuating high-grade pyrexia, and florid described in the literature. (HEPATOLOGY 1997;26:5-8.) psychosis. Bilirubin levels had risen to 622 mmol/L, and alkaline phosphatase had risen to 322 IU/L. Coagulation status
CASE REPORT
was unchanged. Corticosteroids were discontinued, and she was transferred to the National Liver Unit for further evalua-A 21-year-old, previously healthy woman presented with tion. a 1-week history of jaundice, pruritus, pale stools, and dark On transfer, she was icteric, overtly psychotic, and pyrexial urine. Apart from monthly intramuscular injections of the (39ΠC). Scratch marks and hepatomegaly were present. Feaanovulant, medroxyprogesterone acetate, for the previous tures of hepatic decompensation were not demonstrable. year, she had taken no other potentially hepatotoxic drugs.
Laboratory investigations revealed a hemoglobin of 8.9 g/dL, Family and social history were noncontributory, and there albumin of 19 g/dL, international normalized ratio of 1.2, were no background risk factors for the development of acute bilirubin of 266 mmol/L, alkaline phosphatase of 295 IU/L, or chronic liver disease. On admission, she was deeply icteric g-glutamyl transpeptidase of 92 IU/L, alanine transaminase with tender hepatomegaly. There were no stigmata of chronic of 102 IU/L, and aspartate transaminase of 64 IU/L. Kayserliver disease, and she had no lymphadenopathy. Initial inves-Fleischer rings were not present on slit-lamp examination. tigations revealed: hemoglobin, 13.0 g/dL; white cell count, Computerized tomography brain scan and chest x-rays were 4.3 1 10 6 ; platelets, 236 1 10 9 ; international normalized normal. Reticulocyte count was normal at 1.8%, as was lacratio, 1.4; albumin, 43 g/dL; bilirubin, 245 mmol/L; alkaline tate dehydrogenase at 254 U/L, and there was no evidence phosphatase, 198 IU/L (normal, 30-110 IU/L); and aspartate of hemolysis on examination of a peripheral blood smear. transaminase, 108 IU/L (normal, 8-40 IU/L). Serological tests Bone marrow aspirate and biopsy showed normal hematopoifor hepatitis A, B, and C, cytomegalovirus, Epstein-Barr virus, esis and no neoplastic infiltration. Serum angiotensin-conadenovirus, toxoplasmosis, and leptospirosis were negative.
verting enzyme levels were 187 U/L (15-85 U/L). Human Serum mitochondrial, smooth muscle, and antinuclear antiimmunodeficiency virus 1 and 2 serology was negative. Serial bodies were absent, and immunoglobulin (Ig) levels were blood cultures and lumbar puncture were sterile, with a nornormal (IgG Γ
6.0 g/L, IgM Γ΅ 0.3 g/L, and IgA Γ
0.7 g/L). mal cerebrospinal fluid protein (0.2 g/L). A second liver bi-Caeruloplasmin levels were 0.5 mg/L (0.2-0.6 mg/L), urine opsy was performed, which revealed persistent perivenular copper excretion was 49 mg/24 h (normal, Γ΅30 mg), a 1cholestasis and confirmed marked ductopenia with only one antitrypsin levels were 2.73 g/L with a normal band on elecof seven portal tracts containing a normal bile duct. All other trophoresis, and ferritin was 215 mg/L (10-100 mg/L).
portal tracts were devoid of ducts or ductules as confirmed by Abdominal imaging with ultrasonography and computed toabsence of staining with the immunohistochemical epithelial mography scanning confirmed hepatomegaly and showed no marker, Cam 5.2. There were many foamy macrophages other abnormalities. Endoscopic retrograde cholangiography within the tracts, but copper-associated protein was absent showed a normal extra-and intrahepatic biliary system, with in keeping with an acute cholestatic syndrome (Figs. 1 and no evidence of sclerosing cholangitis. Percutaneous liver bi-2). Colonoscopy with biopsy revealed no evidence of colitis.
Over the next 4 weeks, pyrexia and pruritus persisted. Steroid-induced psychosis was successfully treated with oral Abbreviations: HD, Hodgkin's disease; Ig, immunoglobulin.
benzodiazepines. Serum bilirubin levels fluctuated between
From the 1 Liver Unit and Departments of 2 Oncology, 3 Pathology, and 4 Radiology,