Vanishing Bile Duct Syndrome and Hodgkin s Lymphoma: Case Report and Thorough Review of the Literature

Case Report

Ann Hematol Oncol. 2021; 8(9): 1365.

Vanishing Bile Duct Syndrome and Hodgkin’s Lymphoma: Case Report and Thorough Review of the Literature

Keramidas V1#, Tastanis C1#, Tsirogianni K1#, Hytiroglou P2 and Papaioannou M1*

1Hematology Unit, 1st Department of Internal Medicine, AHEPA University Hospital, Medical School, Aristotle University of Thessaloniki, Greece

2Department of Pathology, Medical School, Aristotle University of Thessaloniki, Greece

#Contributed Equally to this Work

*Corresponding author: Papaioannou M, Hematology Unit, 1st Department of Internal Medicine, AHEPA University Hospital, Medical School, Aristotle University of Thessaloniki, S. Kiriakidi 1, 54636, Thessaloniki, Greece

Received: May 26, 2021; Accepted: July 13, 2021; Published: July 20, 2021


Vanishing Bile Duct Syndrome (VBDS) is a rare, acquired disorder, characterized by progressive destruction and loss of intrahepatic bile ducts. The main clinical manifestations are jaundice and pruritus, caused by intralobular cholestasis. Although the pathogenic mechanism is poorly understood, VBDS has been associated with numerous etiologies such as medications, malignancies, infections and autoimmune diseases. This syndrome can appear as a paraneoplastic phenomenon in patients with Hodgkin’s Lymphoma (HL). Diagnosis is based on clinical evaluation and confirmed via liver biopsy, while treating the underlying cause is the main therapeutic target. If bile duct regeneration does not occur, possible outcomes include cirrhosis, hepatic failure and death, with liver transplantation being the only curative option.

In this paper, we describe a case of HL-related VBDS in a 31-year-old female patient, who presented with jaundice, pruritus and cervical lymphadenopathy. The stage of HL was determined as IIA and a liver biopsy was performed, which confirmed the degeneration of bile ducts. The patient was treated with the ABVD regimen and dexamethasone. Follow-up tests were normal and supported the full remission hypothesis. We conducted an analytical literature review and collected the available data from 38 confirmed cases, regarding the epidemiology, viral infections, clinical findings, therapeutic options and outcome.

Keywords: Vanishing bile duct syndrome; Hodgkin’s lymphoma; Paraneoplastic cholestasis; Jaundice


Vanishing Bile Duct Syndrome (VBDS) refers to a group of acquired disorders characterized primarily by cholestasis as a result of the progressive destruction and disappearance of the intrahepatic bile ducts. Although the pathogenetic mechanisms are not fully understood, VBDS has been associated with multiple etiologies including autoimmune diseases, infections, medications, neoplastic disorders and genetic abnormalities. VBDS can be a paraneoplastic manifestation of Hodgkin’s Lymphoma (HL), which typically presents with jaundice, pruritus and weight loss. This paper aims to report a case of HL-related VBDS and review the existing literature.

Case Presentation

A 31-year-old woman was admitted to the hematology department with jaundice, pruritus and cervical lymphadenopathy of one month. Her past medical history is significant for heterozygous beta-thalassemia. Physical examination was repeated and confirmed the existence of jaundice and cervical lymphadenopathy. Her abnormal lab tests were: bilirubin 21.5mg/dl [n.v. <0.9mg/dl], direct 13.7mg/dl [<0.3mg/dl], LDH 1177U/L [<240U/l], SGOT 205U/L [<32U/l], SGPT 258U/L [<33U/l], ALP 1041U/L [35-104 U/l], γ-GT 458U/L [5-36 U/l], albumin 2.49g/dl [3.5-5.2 g/dl], CRP 6.49mg/dl [<0.5mg/dl], B2 microglobulin 5.28mg/L [<2.5mg/L]. Blood counts revealed leukocytosis (17.240/μL), thrombocytosis (701.000/μL), ESR 140mm and anemia (Hb 7.3g/dL). Viral hepatitis panel, HIV and Toxoplasma antibody tests were negative. However, EBV and CMV antibody tests were positive indicating a past infection. Remaining laboratory data including tumor markers and A1-antitrypsin were within normal limits.

Abdominal ultrasound examination did not reveal any pathological findings. The cervical lymphadenopathy required further investigation, so a biopsy was performed. HL (nodular sclerosis) was confirmed. A Computerized Tomography (CT) of the thorax and abdomen and a bone marrow biopsy was conducted to determine the stage of HL. Enlarged lymph nodes were detected at the mediastinum whereas the abdominal region was free of pathological findings and bone marrow biopsy was found to be normal. Keeping in mind that an abdominal CT scan is unable to detect intrahepatic filtration, a liver biopsy was performed (Figure 1). For that purpose, the levels of bilirubin needed to be reduced, so the patient was treated with dexamethasone (40mg/day for 4 days). The+ liver biopsy revealed medium to severe centrilobular cholestasis, no neoplastic cells and degenerative alteration of bile ducts with more than 50% being absent in a specimen consisting of 10 portal triads. The stage of HL was IIA and VBDS was diagnosed. ABVD, the initial chemotherapy regimen for newly diagnosed early-stage HL, was administered to the patient, with half the dose of anthracycline for the first cycle and full dose thereafter.