Intrahepatic Cholestasis Related to Vanishing Bile Duct Syndrome in Hodgkin's Disease

Gottrand, Frédéric; Çullu, Fügen; Mazingue, Françoise; Nelken, Brigitte; Lecomte-Houcke, M; Farriaux, J. P.

doi:10.1097/00005176-199704000-00013

Cited by 22 publications

(17 citation statements)

References 13 publications

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“…Among these, one case had repeated liver biopsy owing to new liver nodules being found, which disclosed involvement of the liver in Hodgkin's disease [3]. Eight cases showed persistent ductopenia and four of them met with mortality due to sepsis, progressive liver failure, or HL [3–9]. Three cases showed ductal regeneration and all remain alive to date [[10,11], and this case] (Table 1).…”

Section: Discussionmentioning

confidence: 99%

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Hodgkin's lymphoma‐related vanishing bile duct syndrome: A case report and literature review

Wong

Chang

et al. 2013

The Kaohsiung J of Med Scie

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We report the case of a 38-year-old man who developed vanishing bile duct syndrome in association with Hodgkin's lymphoma. He was noted to have cervical lymphadenopathy and marked elevation of total serum bilirubin at diagnosis. He achieved complete remission with normalization of serum bilirubin after eight courses of Adriamycin, bleomycin, vinblastine, and dacarbazine chemotherapy followed with autologous hematopoietic cell transplantation. Consecutive liver biopsies performed at diagnosis and at the stage of complete remission revealed the disappearance and regeneration of interlobular bile ducts, respectively. Our case provides pathological evidence that Hodgkin's lymphoma-related vanishing bile duct syndrome is a reversible bile duct injury disease. Bilirubin is a reliable serum marker to monitor the treatment response of these cases. The mechanism to develop hyperbilirubinemia with vanishing bile duct in such a case of Hodgkin's lymphoma remains to be studied. A literature review was carried out.

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Section: Discussionmentioning

confidence: 99%

“…Following Hubscher et al's work, we performed a literature search in PubMed database using “vanishing bile duct syndrome”, “Hodgkin's lymphoma”, and “ductopenia” as keywords. The search yielded a total of 23 HL‐related VBDS cases from 1993 to 2011 (Table 1) [3–19], which included both English and non‐English publications.…”

Section: Discussionmentioning

confidence: 99%

Hodgkin's lymphoma‐related vanishing bile duct syndrome: A case report and literature review

Wong

Chang

et al. 2013

The Kaohsiung J of Med Scie

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“…Nearly all previously reported patients died of liver failure, despite treatment-induced remission of Hodgkin's lymphoma [13][14][15]. Only Crosbie et al described a single patient with successfully treated lymphoma who survived with serum bilirubin levels decreasing from 622 to 11 mmol/l.…”

Section: Discussionmentioning

confidence: 99%

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

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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.

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“…[15]1NoNoNoRespiratory arrestBirrer M.J. [16]1YesFullUnknownSepsisHubscher S.G. [1]3YesReducedNoHepatic failureJansen P.L.M. [17]1YesFullLateHepatic failureWarner A.S. [18]1YesFullNoCureGottrand F. [19]1NoNoNoHepatic failureCrosbie O.M. [20]1YesFullYesCureDe Medeiros B.C.…”

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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Intrahepatic Cholestasis Related to Vanishing Bile Duct Syndrome in Hodgkin's Disease

Cited by 22 publications

References 13 publications

Hodgkin's lymphoma‐related vanishing bile duct syndrome: A case report and literature review

Hodgkin's lymphoma‐related vanishing bile duct syndrome: A case report and literature review

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

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

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