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.