In general, Hodgkin’s disease is a lymph node-based disease, which usually spreads along the lymphatic chain to contiguous lymph node areas. Liver involvement in Hodgkin’s disease depends on its stage – it is rare in early phase and common in end-stage of this disease. Consequently, less than 4% of patients with newly identified Hodgkin’s disease have significant hepatic symptoms (1). The liver was infiltrated with atypical lymphoid cells in 5-10% of cases in biopsy studies and in up to 50% of cases at autopsies (2, 3). Only occasionally the initial clinical presentation of Hodgkin’s disease comes from the liver. In such cases this hematological disease may imitate the primary liver or biliary tract disease. We present an unusual case of Hodgkin’s disease that started as cholestatic hepatopathy caused by both the granulomatous hepatitis and the stenosis of extrahepatic biliary duct.

A 48-year old man was admitted to local hospital because of cholestasis, loss of weight (20 kg over 6 months), night sweats and fever (March, 2011). He denied any abdominal pain. Four years earlier he underwent surgery due to perforation of small bowel and peritonitis secondary to blunt abdominal trauma. There was no history of viral hepatitis and he was not taking any medication. The patient was a farmer specialized in fruit growing. Each day he was smoking approximately 20 cigarettes and was drinking 3 cups of coffee.

An abdominal ultrasonography showed hepatosplenomegaly (the longitudinal span of the right hepatic lobe was 15 cm and that of spleen 13 cm). In the right hepatic lobe tumor-like lesions with the greatest diameter of 4 cm were visualized.

An abdominal computed tomography (CT) scan demonstrated enlarged and heterogenous liver with disseminated hypovascular focal lesions located in the segments 2, 5, 6 and 8. The spleen was enlarged and contained solid focal lesions suggestive of metastases (fig. 1). Besides, CT demonstrated multiple retroperitoneal and hepatic perihilar lymph nodes 10-13 mm in size. The pancreas was of normal size without focal lesions. An endoscopic retrograde cholangiopancreatography (ERCP) showed stenosis of the hepatic biliary duct at the level of the cystic duct. The intrahepatic biliary ducts were moderately dilated. The plastic stent 10 F 12 cm was inserted through the stenosis. It resulted in a decrease of serum bilirubin level from 4.5 to 0.8 mg/dl.

The liver biopsy showed features of granulomatous hepatitis with no evidence of malignant disease (April, 2011). The patient was referred to our department with strong suspicion of cholangiocarcinoma (July, 2011). The physical examination confirmed hepatosplenomegaly and disclosed mild bilateral enlargement of axillary (size 1-1.5 cm) and inguinal (size 1 cm) lymph nodes. The patient was febrile and used antipyretics. The administration of corticosteroids (prednisone 40 mg) justified by hepatic histology stopped the fever immediately. Relevant laboratory findings included: total bilirubin 3.2 mg/dl, alkaline phosphatase; ALP 622 IU/l (upper reference value 120 IU/l), gama-glutamyltranspetidase; GGTP 646 IU/l (upper reference value 55 IU/l), alanine aminotransferases; ALT 118 IU/l (upper reference value 45 IU/l), aspartate aminotransferases; AST 86 IU/l (upper reference value 35 IU/l) and C-reactive protein; CRP 74.6 mg/l (upper reference value 5 mg/l). Serological examinations for hepatitis A virus, hepatitis B virus, hepatitis C virus, human immunodeficiency virus and Epstein?Barr virus were negative. Tests for HCV RNA, antinuclear antibodies, antimitochondrial antibodies and anti-smooth muscle antibodies were also negative. The serum level of CA19-9 was 71.8 IU/l (upper reference value 39 IU/l). The Quantiferon test was negative.

During hospital follow-up the left-side anisocoria has occurred. For this reason the computed tomography of the brain and the chest radiography were performed. These examinations did not reveal any significant pathology.