Vanishing Bile Duct Syndrome Arising  in a Patient With T-Cell–Rich Large  B-Cell Lymphoma

Gagnon, Mona-Josée; Nguyen, Bich; Olney, Harold J.; Lemieux, Bernard

doi:10.1200/jco.2012.46.7787

Cited by 9 publications

(6 citation statements)

References 13 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…[38]1YesFullNoCureUmit H. [39]1UnknownUnknownUnknownUnknownGill R.M. [40]1YesReducedNoHepatic failureForamiti S. [41]1YesFullYesHepatic failureGagnon MF [42]1YesFullNoCureWong KM [43]1YesFullNoCureAleem A1 [44]1YesFullNoHepatic failureNader K [45]1YesFullNoHepatic failureOur patient1YesFullYesCure*either chemotherapy or radiotherapy.…”

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

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

show abstract

“… 1, 2 VBDS is a rare cause of jaundice in patients with Hodgkin’s and non-Hodgkin’s diseases such as ALCL, a sub-type of lymphoma primarily involving CD30-positive T cells. 3, 4 The overall prognosis remains poor, and treatment is dependent on the underlying etiology, with the emphasis on supportive care. The loss of bile ducts is often an irreversible process; however, the resolution following successful chemotherapy in lymphoma patients has been reported.…”

Section: Case Reportmentioning

confidence: 99%

Anaplastic Large Cell Lymphoma and Vanishing Bile Duct Syndrome

Sreepati

Liangpunsakul

2016

ACG Case Reports Journal

View full text Add to dashboard Cite

A 39-year-old healthy man noticed a painful left chest wall mass associated with daily fevers, generalized pruritus, and jaundice. A hepatic panel showed a total bilirubin of 24.9 mg/dL, AST of 224 U/L, ALT of 506 U/L, and alkaline phosphatase of 1947 U/L. A chest computerized tomography (CT) showed a 9.6 x 5.2 cm chest wall mass extending to the mediastinum, and biopsy revealed anaplastic large cell lymphoma (ALCL). An abdominal CT showed normal liver parenchyma without ductal obstruction. Liver biopsy revealed bile duct damage, cholestasis, and hepatocyte injury ( Figure 1).He underwent chemotherapy with gemcitabine, dexamethasone, and platinum. However, his jaundice and pruritus worsened, and a repeat liver biopsy demonstrated the complete loss of intrahepatic bile ducts, compatible with vanishing bile duct syndrome (VBDS; Figure 2). Eight weeks after chemotherapy, he is free of malignancy with complete resolution of the chest wall mass. He is fully active but remains jaundiced with a total bilirubin of 29.7 mg/dL. VBDS is an acquired disorder resulting in progressive destruction and disappearance of the intrahepatic bile ducts. It can be the manifestation of congenital disease, neoplastic disorders, or drug-induced liver injury .1,2 VBDS is a rare cause of jaundice in patients with Hodgkin's and non-Hodgkin's diseases such as ALCL, a sub-type of lymphoma primarily involving CD30-positive T cells. 3,4 The overall prognosis remains poor, and treatment is dependent on the underlying etiology, with the emphasis on supportive care. The loss of bile ducts is often an irreversible process; however, the resolution following successful chemotherapy in lymphoma patients has been reported. 5 Liver transplantation should be considered for those with decompensation if the underlying disease is under control.

show abstract

“…In our case, the level of marrow involvement was stable and minimal (at most 2% of total cellularity). In addition, VBDS arising in non‐Hodgkin lymphoma (NHL) is rare with only three reported cases . Pathologies of NHL are mostly high‐grade, including T‐cell‐rich large B‐cell lymphoma, T‐cell lymphoma not otherwise specified, and stage IV B‐cell NHL.…”

Section: Liver Function Tests At Initial Presentation Through To Diagmentioning

confidence: 99%

“…In addition, VBDS arising in non-Hodgkin lymphoma (NHL) is rare with only three reported cases. [10][11][12] Pathologies of NHL are mostly highgrade, including T-cell-rich large B-cell lymphoma, T-cell lymphoma not otherwise specified, and stage IV B-cell NHL. Although the possibility of coincident MZL cannot be excluded, it appears unlikely to account solely for the severity and large variation in the patient's symptoms given the minor and stable marrow involvement over time.…”

Section: Brief Communicationsmentioning

confidence: 99%

Vanishing bile duct syndrome and immunodeficiency preceding the diagnosis of Hodgkin lymphoma

Yeh

Lokan

Grigg

2014

Internal Medicine Journal

View full text Add to dashboard Cite

Vanishing bile duct syndrome (VBDS) in association with Hodgkin lymphoma (HL) is well described but not well understood. We report an unusual case of a 75-year-old patient presenting with biopsy-proven VBDS and immunodeficiency, without identifiable cause, which showed a waxing and waning course, culminating in the development of HL 18 months later. To our knowledge, this is the first adult case in which VBDS preceded the diagnosis of HL by such a long period.

show abstract

Vanishing Bile Duct Syndrome Arising in a Patient With T-Cell–Rich Large B-Cell Lymphoma

Cited by 9 publications

References 13 publications

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

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

Anaplastic Large Cell Lymphoma and Vanishing Bile Duct Syndrome

Vanishing bile duct syndrome and immunodeficiency preceding the diagnosis of Hodgkin lymphoma

Contact Info

Product

Resources

About