Skip to main content

Thank you for visiting nature.com. You are using a browser version with limited support for CSS. To obtain the best experience, we recommend you use a more up to date browser (or turn off compatibility mode in Internet Explorer). In the meantime, to ensure continued support, we are displaying the site without styles and JavaScript.

  • Case Study
  • Published:

A case of vanishing bile duct syndrome and IBD secondary to Hodgkin's lymphoma

Nature Clinical Practice Gastroenterology & Hepatology volume 5, pages 49–53 (2008)Cite this article

Abstract

Background 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.

Investigations Physical examination, laboratory investigations, abdominal ultrasound, CT scans, magnetic resonance cholangiopancreatography, endoscopic retrograde cholangiopancreatography, colonoscopies with biopsies, hepatic biopsy, axillary lymph node biopsy.

Diagnosis Hodgkin's lymphoma with secondary vanishing bile duct syndrome and IBD, type unclassified.

Management The initial symptoms were managed with budesonide, but following recurrence, the patient's underlying lymphoma was treated with nitrogen mustard and dexamethasone.

This is a preview of subscription content, access via your institution

Access options

Buy this article

  • Purchase on Springer Link
  • Instant access to full article PDF
Buy now

Prices may be subject to local taxes which are calculated during checkout

Figure 1: Vanishing bile duct syndrome.
Figure 2: IBD, type unclassified.
Figure 3: The budesonide effect.
Figure 4: Delayed lymphadenopathy.

References

  1. Reese GE et al. (2006) Diagnostic precision of anti-Saccharomyces cerevisiae antibodies and perinuclear antineutrophil cytoplasmic antibodies in inflammatory bowel disease. Am J Gastroenterol 101: 2410–2422

    Article  CAS  Google Scholar 

  2. Satsangi J et al. (2006) The Montreal classification of inflammatory bowel disease: controversies, consensus, and implications. Gut 55: 749–753

    Article  CAS  Google Scholar 

  3. Koren G et al. (1992) The effects of impaired liver function on the elimination of antineoplastic agents. Ann Pharmacother 26: 363–371

    Article  CAS  Google Scholar 

  4. Hubscher SG et al. (1993) Vanishing bile duct syndrome: a possible mechanism for intrahepatic cholestasis in Hodgkin's lymphoma. Hepatology 17: 70–77

    Article  CAS  Google Scholar 

  5. Crosbie OM et al. (1997) Resolution of paraneoplastic bile duct paucity following successful treatment of Hodgkin's disease. Hepatology 26: 5–8

    Article  CAS  Google Scholar 

  6. Cavalli G et al. (1979) Changes in the small biliary passages in the hepatic localization of Hodgkin's disease. Virchows Arch A Pathol Anat Histol 384: 295–306

    Article  CAS  Google Scholar 

  7. Lefkowitch JH et al. (1985) Hepatic Hodgkin's disease simulating cholestatic hepatitis with liver failure. Arch Pathol Lab Med 109: 424–426

    CAS  PubMed  Google Scholar 

  8. Gottrand F et al. (1997) Intrahepatic cholestasis related to vanishing bile duct syndrome in Hodgkin's disease. J Pediatr Gastroenterol Nutr 24: 430–433

    Article  CAS  Google Scholar 

  9. Yusuf MA et al. (2000) Jaundice caused by vanishing bile duct syndrome in a child with Hodgkin lymphoma. J Pediatr Hematol Oncol 22: 154–157

    Article  CAS  Google Scholar 

  10. Loftus EV Jr et al. (2000) Risk of lymphoma in inflammatory bowel disease. Am J Gastroenterol 95: 2308–2312

    Article  Google Scholar 

  11. Lewis JD et al. (2001) Inflammatory bowel disease is not associated with an increased risk of lymphoma. Gastroenterology 121: 1080–1087

    Article  CAS  Google Scholar 

  12. Ramasamy KA et al. (2002) A case of primary non-Hodgkin's lymphoma of the transverse colon presenting as inflammatory bowel disease. Eur J Gastroenterol Hepatol 14: 1401–1403

    Article  Google Scholar 

  13. Vincenzi B et al. (2001) Unusual presentation of Hodgkin's disease mimicking inflammatory bowel disease. Leuk Lymphoma 42: 521–526

    Article  CAS  Google Scholar 

  14. Scheuer PJ (1998) Ludwig Symposium on biliary disorders—part II. Pathologic features and evolution of primary biliary cirrhosis and primary sclerosing cholangitis. Mayo Clin Proc 73: 179–183

    Article  CAS  Google Scholar 

  15. Hamid S et al. (1997) Protracted cholestatic hepatitis after the use of prostata. Ann Intern Med 127: 169–170

    Article  CAS  Google Scholar 

  16. Barta SK et al. (2006) Idiopathic cholestasis as a paraneoplastic phenomenon in Hodgkin's lymphoma. Clin Lymphoma Myeloma 7: 77–82

    Article  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. AT DeBenedet is a second-year Resident Physician in the Department of Internal Medicine, University of Michigan Health System, Ann Arbor, Michigan, USA.,

    Anthony T DeBenedet

  2. and PG Northup is an Assistant Professor in the Department of Internal Medicine, Division of Gastroenterology and Hepatology, KB Enfield is a first-year fellow in the Department of Internal Medicine, Division of Pulmonary and Critical-Care Medicine, and RL Woodford is a second-year Resident Physician in the Department of Pathology, CL Berg is an Associate Professor and Interim Chief of Gastroenterology and Hepatology, University of Virginia Health System, Charlottesville, Virginia, USA.,

    Carl L Berg, Kyle B Enfield, Randall L Woodford & Patrick G Northup

  3. AK Bennett is a Staff Pathologist at Bon Secours St Mary's Hospital, Richmond, Virginia.,

    Audrey K Bennett

Authors
  1. Anthony T DeBenedet
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Carl L Berg
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Kyle B Enfield
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Randall L Woodford
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Audrey K Bennett
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Patrick G Northup
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Anthony T DeBenedet.

Ethics declarations

Competing interests

The authors declare no competing financial interests.

Rights and permissions

Reprints and permissions

About this article

Cite this article

DeBenedet, A., Berg, C., Enfield, K. et al. A case of vanishing bile duct syndrome and IBD secondary to Hodgkin's lymphoma. Nat Rev Gastroenterol Hepatol 5, 49–53 (2008). https://doi.org/10.1038/ncpgasthep1001

Download citation

  • Received:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1038/ncpgasthep1001

This article is cited by

Search

Advanced search

Quick links

Nature Briefing

Sign up for the Nature Briefing newsletter — what matters in science, free to your inbox daily.

Get the most important science stories of the day, free in your inbox. Sign up for Nature Briefing