Cholestatic Liver Disease with Ductopenia (Vanishing Bile Duct Syndrome) in Hodgkin's Disease: Report of a Case

Güliter, Sefa; Erdem, Özlem; Işık, Mine; Yamaç, Kadri; Uluoğlu, Ömer

doi:10.1177/030089160409000516

Cited by 21 publications

(19 citation statements)

References 17 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Including our patient, 18 cases with this complication have been reported [5][6][7][8][9]. As in our patient, most cases presented with jaundice and Hodgkin's lymphoma was diagnosed later.…”

Section: Discussionmentioning

confidence: 51%

Severe jaundice, due to vanishing bile duct syndrome, as presenting symptom of Hodgkinʼs lymphoma, fully reversible after chemotherapy

Leeuwenburgh¹,

Lugtenburg

Buuren³

et al. 2008

European Journal of Gastroenterology & Hepatology

View full text Add to dashboard Cite

Liver involvement in Hodgkin's lymphoma is common and is caused by hepatic infiltration, biliary obstruction by lymphoma, hepatitis, sepsis or complications of chemotherapeutic treatment. Jaundice caused by the vanishing bile duct syndrome related to Hodgkin's lymphoma is very rare. The mechanism is poorly understood but a paraneoplastic effect seems most likely as liver biopsy samples show cholestasis in the absence of lymphoma cells. Despite adequate treatment almost all reported patients died of liver failure or disease progression. Disease progression is explained partly by the difficulties encountered in the administration of potential hepatotoxic chemotherapy in severely cholestatic patients. We describe a 17-year-old man with vanishing bile duct syndrome and Hodgkin's lymphoma who was treated successfully with chemotherapy. The markedly elevated serum bilirubin levels completely normalized. Our case demonstrates that although dosing of chemotherapy in this situation can be very difficult, a good clinical outcome is possible, which makes the attempt at curative treatment worthwhile.

show abstract

Section: Discussionmentioning

confidence: 51%

Severe jaundice, due to vanishing bile duct syndrome, as presenting symptom of Hodgkinʼs lymphoma, fully reversible after chemotherapy

Leeuwenburgh¹,

Lugtenburg

Buuren³

et al. 2008

European Journal of Gastroenterology & Hepatology

View full text Add to dashboard Cite

show abstract

“…Cholestasis can be caused by direct infiltration of lymphoma cells, extrahepatic biliary obstruction, viral hepatitis, drug hepatotoxicity, or vanishing bile duct syndrome [89–91]. Approximately 3%–13% of patients with HL present with jaundice [90]. Acute liver failure can be caused by ischemia secondary to compression of the hepatic sinusoids by infiltrating lymphoma cells [92, 93].…”

Section: Lymphoid Neoplasmsmentioning

confidence: 99%

Hepatic Manifestations in Hematological Disorders

Murakami

Shimizu

2013

International Journal of Hepatology

View full text Add to dashboard Cite

Liver involvement is often observed in several hematological disorders, resulting in abnormal liver function tests, abnormalities in liver imaging studies, or clinical symptoms presenting with hepatic manifestations. In hemolytic anemia, jaundice and hepatosplenomegaly are often seen mimicking liver diseases. In hematologic malignancies, malignant cells often infiltrate the liver and may demonstrate abnormal liver function test results accompanied by hepatosplenomegaly or formation of multiple nodules in the liver and/or spleen. These cases may further evolve into fulminant hepatic failure.

show abstract

“…[3]1YesFullYesCureOzkan A. [26]1NoNoLateHepatic failureRipoll C. [27]2YesFullNoHepatic failureYesFullYesCureKomurcu S. [28]1YesFullNoHepatic failureLiangpunsakul S. [29]1YesFullNoCureGuliter S. [30]1YesFullLateHepatic failureCórdoba Iturriagagoitia A. [31]1YesFullNoCureHan W.S.…”

Section: Resultsmentioning

confidence: 99%

Complete remission of paraneoplastic vanishing bile duct syndrome after the successful treatment of Hodgkin’s lymphoma: a case report and review of the literature

et al. 2014

View full text Add to dashboard Cite

BackgroundVanishing bile duct syndrome has been associated with different pathologic conditions (adverse drug reactions, autoimmune diseases, graft versus host disease, and cancer). Though its causes are unknown, an immune-related pathogenesis is the most likely one. Vanishing bile duct syndrome can evolve to hepatic failure and, eventually, to death. The treatment is uncertain, but it needs the resolution of the underlying pathologic condition.Case presentationWe describe the association of Hodgkin’s lymphoma with a syndrome characterized by cholestasis, aminotransferase elevation and an histological picture of bile duct loss. All other causes of hepatic function impairment were excluded (in particular, drugs, viral and autoimmune related diseases) eventually leading to the diagnosis of vanishing bile duct syndrome. Despite the fact that the dysfunction is not caused by hepatic Hodgkin’s lymphoma involvement, liver impairment can limit the optimal therapy of Hodgkin’s lymphoma. A treatment consisting of ursodeoxycholic acid, prednisone, and full dose chemotherapy restored hepatic function and achieved complete and long-lasting remission of Hodgkin’s lymphoma.ConclusionWe reviewed all case reports showing that vanishing bile duct syndrome is a dismal paraneoplastic syndrome being fatal in a high proportion of patients if not adequately treated. Indeed, this syndrome requires both an early recognition and an appropriate aggressive treatment consisting of full dose upfront chemotherapy which is the only way to achieve a resolution of the vanishing bile duct syndrome. Delayed or reduced intensity treatments unfavorably correlate with survival.

show abstract

Cholestatic Liver Disease with Ductopenia (Vanishing Bile Duct Syndrome) in Hodgkin's Disease: Report of a Case

Cited by 21 publications

References 17 publications

Severe jaundice, due to vanishing bile duct syndrome, as presenting symptom of Hodgkinʼs lymphoma, fully reversible after chemotherapy

Severe jaundice, due to vanishing bile duct syndrome, as presenting symptom of Hodgkinʼs lymphoma, fully reversible after chemotherapy

Hepatic Manifestations in Hematological Disorders

Complete remission of paraneoplastic vanishing bile duct syndrome after the successful treatment of Hodgkin’s lymphoma: a case report and review of the literature

Contact Info

Product

Resources

About