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

DeBenedet, Anthony T.; Berg, Carl L.; Enfield, Kyle B.; Woodford, Randall L.; Bennett, Audrey K.

doi:10.1038/ncpgasthep1001

Cited by 11 publications

(9 citation statements)

References 16 publications

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“…The disappearance of the bile ducts is a common mechanism for different diseases due to multiple pathologies 2. Conditions which initiate this kind of bile duct destruction could be congenital, idiopathic, toxin or drug induced, infectious, neoplastic or immunologic 3–5. In our patient, there was no evidence of any infectious or neoplastic process as evidenced by the serological testing, viral amplification methods and the biopsy.…”

Section: Discussionmentioning

confidence: 58%

Vanishing bile duct syndrome in a HIV patient on HAART therapy

2011

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Section: Discussionmentioning

confidence: 58%

Vanishing bile duct syndrome in a HIV patient on HAART therapy

2011

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“…[33]1NoNoNoHepatic failureBarta S.K. [34]1YesFullNoCureDeBenedet A.T. [35]1YesReducedNoHepatic failureLeeuwenburgh I. [36]1YesReducedNoCurePass A.K.…”

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

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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.

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“…Chemotherapy with nitrogen mustard agents and steroids may be considered in the setting of liver failure, and there have been successful reports with this approach in HL patients with hepatic dysfunction [11,15]. This treatment approach was employed for our patient, with marked improvement initially in his liver and renal dysfunction.…”

Section: An Unusual Presentation Of Liver Failure In a Patient With Pmentioning

confidence: 91%

“…Primary GI HL involving the bowel has been associated with inflammatory bowel disease (IBD) in several case reports [9][10][11][12]. Kumar et al reported 4 cases of GI HL with concurrent EBV infection, with two of the cases occurring in patients with Crohn's disease.…”

Section: An Unusual Presentation Of Liver Failure In a Patient With Pmentioning

confidence: 99%

An Unusual Presentation of Liver Failure in a Patient with Primary Gastrointestinal Hodgkin's Lymphoma

et al. 2011

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Introduction. Hodgkin's lymphoma (HL) presenting either with primary bowel involvement or with cholestasis is unusual. The combination of primary gastrointestinal HL presenting with cholestasis and ductopenia has not been previously described. Case Report. We present a case of primary gastrointestinal HL with evidence of liver involvement, but also with prominent ductopenia on liver biopsy and associated intrahepatic cholestasis. A 50-year-old man with a history of Crohn's disease presented with a bowel obstruction, for which he underwent a small bowel resection. Histology revealed HL. His course was complicated by cholestatic liver failure. A subsequent liver biopsy revealed both focal involvement by lymphoma and ductopenia, resembling vanishing bile duct syndrome (VBDS). He was treated with chemotherapy with improvement in his cholestasis, but he eventually succumbed due to further complications of his disease and treatment toxicities. Conclusion. This case of primary gastrointestinal HL associated with ductopenia does not meet classic criteria for VBDS, but the clinical presentation and pathology are suggestive of a VBDS-like paraneoplastic process. Therapies for HL in the setting of cholestatic liver failure require special consideration, but some reports of durable remissions and recovery of liver function have been reported.

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

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

Cited by 11 publications

References 16 publications

Vanishing bile duct syndrome in a HIV patient on HAART therapy

Vanishing bile duct syndrome in a HIV patient on HAART therapy

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

An Unusual Presentation of Liver Failure in a Patient with Primary Gastrointestinal Hodgkin's Lymphoma

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