Case Report
Idiopathic Cholestasis as a Paraneoplastic Phenomenon in Hodgkin's Lymphoma

https://doi.org/10.3816/CLM.2006.n.044Get rights and content

Abstract

Paraneoplastic cholestasis is an uncommon presenting symptom in Hodgkin's lymphoma (HL). Two distinct entities causing this clinical picture have been described: idiopathic cholestasis and vanishing bile duct syndrome (VBDS). We report a patient with idiopathic cholestasis and early-stage HL with favorable risk factors whose liver biopsy results were consistent with intrahepatic cholestasis and no ductopenia. Other causes for cholestatic jaundice were ruled out. He was treated with subtotal lymphoid irradiation and subsequently experienced a steady improvement in his liver function tests to near normal over 21 months. This case illustrates a rare paraneoplastic phenomenon, and a review of the available literature is included. We also discuss the differences between HL-related idiopathic cholestasis and VBDS. The distinction between these 2 diseases has prognostic implications: idiopathic cholestasis is usually reversible in early-stage HL after treatment, whereas patients who develop VBDS commonly die from their disease. There is no established approach to the management of paraneoplastic cholestasis. We postulate that single-modality radiation therapy alone should be considered a valid treatment alternative in early-stage HL with idiopathic cholestasis.

References (44)

  • NH Dich et al.

    Hepatic involvement in Hodgkin's disease

    Cancer

    (1989)
  • DL Thiele

    Hepatic manifestations of systemic disease and other disorders of the liver

  • BA Bouroncle et al.

    Pathogenesis of jaundice in Hodgkin's disease

    Arch Intern Med

    (1962)
  • C Groth et al.

    Diagnosis of malignant lymphoma at laparotomy disclosing intrahepatic cholestasis

    Acta Chir Scand

    (1972)
  • DR Perrera et al.

    Cholestasis associated with extrabiliary Hodgkin's disease

    Gastroenterology

    (1974)
  • PN Trewby et al.

    Liver disease as presenting manifestation of Hodgkin's disease

    Quart J Med

    (1979)
  • DA Lieberman

    Intrahepatic cholestasis due to Hodgkin's disease: an elusive diagnosis

    J Clin Gastroenterol

    (1986)
  • AS Warner et al.

    Extrahepatic Hodgkin's disease and cholestasis

    Am J Gastroenterol

    (1994)
  • PL Jansen et al.

    Intrahepatic cholestasis and biliary cirrhosis associated with extrahepatic Hodgkin's disease

    Neth J Med

    (1994)
  • S Yalcin et al.

    Extrahepatic Hodgkin's disease with intrahepatic cholestasis: report of 2 cases;

    Oncology

    (1999)
  • S Liangpunsakul et al.

    Hodgkin's disease presenting as cholestatic hepatitis with prominent ductal injury

    Eur J Gastroenterol Hepatol

    (2002)
  • SG Hubscher et al.

    Vanishing bile duct syndrome: a possible mechanism for intrahepatic cholestasis in Hodgkin's lymphoma

    Hepatology

    (1993)
  • Cited by (25)

    View all citing articles on Scopus

    Electronic forwarding or copying is a violation of US and International Copyright Laws.

    Authorization to photocopy items for internal or personal use, or the internal or personal use of specific clients, is granted by CIG Media Group, LP, ISSN #1557-9190, provided the appropriate fee is paid directly to Copyright Clearance Center, 222 Rosewood Drive, Danvers, MA 01923 USA 978-750-8400.

    View full text