Vanishing bile duct syndrome in Hodgkin's disease: case report

Rossini, Marta Soares; Lorand-Metze, Irene; Oliveira, Gislaine B.; Souza, Cármino Antônio de

doi:10.1590/s1516-31802000000500008

Cited by 22 publications

(15 citation statements)

References 10 publications

(17 reference statements)

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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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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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“…[24]1NoNoLateHepatic failureRossini M.S. [25]1YesFullNoHepatic failureAllory Y. [3]1YesFullYesCureOzkan A.…”

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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“…In addition to cases of liver rejection following transplantation, PBC and PSC, VBDS has also been reported in cases of Hodgkin's disease, hystiocytosis X, and adult non‐Hodgkin's lymphoma [1,3,4,13,14]. To the best of our knowledge, the above presentation is the first reported case of VBDS in a patient with HIV or AIDS and should be considered as a potential aetiology for chronic intrahepatic cholestasis in such patients.…”

Section: Discussionmentioning

confidence: 97%

Vanishing bile duct syndrome in a patient with advanced AIDS

Hindupur¹,

Yeung²,

Shroff³

et al. 2007

HIV Medicine

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A 39-year-old HIV-infected woman developed signs and symptoms of obstructive jaundice and cholestasis. Serological tests were positive for cytomegalovirus (CMV) infection. There was no evidence of AIDS cholangiopathy in ultrasonography or magnetic resonance cholangiopancreatography (MRCP). A liver biopsy revealed marked ductopenia and the patient was diagnosed with vanishing bile duct syndrome, thought to be secondary to CMV infection as a result of profound immunosuppression. To the best of our knowledge, this is the first reported case of vanishing bile duct syndrome diagnosed in a patient with HIV/AIDS.Keywords: ductopenia, idiopathic adulthood ductopenia, intrahepatic cholestasis, vanishing bile duct syndrome Case reportA 39-year-old woman presented with a complaint of fatigue, anorexia, nausea and vomiting, of duration 3 days. She also reported a 1-week history of scleral icterus, pruritis and abdominal pain. The nausea and vomiting were described as nonbilious and nonbloody, occurring three times a day, while the abdominal pain was localized to the right upper quadrant without radiation or association with meals. Past medical history was significant for HIV infection and hepatitis C virus (HCV) infection diagnosed in 1997. She was not taking any medication, including highly active antiretroviral therapy (HAART) or treatment for HCV infection, because of a history of polysubstance abuse of cocaine, alcohol and intravenous drugs. Her last documented CD4 cell count and quantitative HIV-1 RNA viral load 7 months prior to admission were 22 cells/mL (3%) and 48 026 HIV RNA copies/mL, respectively. Her weight was 43 kg and all initial vital signs were normal.Physical examination was significant for cachexia and generalized icterus. The right upper quadrant was tender to palpation without rebound tenderness or guarding. There were no masses palpated and no evidence of ascites. Murphy's sign was negative. The liver span was measured as 5 cm. No cutaneous manifestations of chronic liver disease were noted. Initial chemistry values were significant for the following: albumin 5 2.9 g/dL, total bilirubin 5 15.4 mg/dL, direct bilirubin 5 11.0 mg/dL, alkaline phosphatase 5 2200 IU/L, aspartate transaminase 5 114 IU/ L and alanine transaminase 5 29 IU/L. The partial thromboplastin time and international normalized ratio were 39 s and 1.4, respectively. Urine analysis revealed proteinuria and bilirubinuria while the CD4 cell count and HIV-1 RNA viral load were measured as 7 cells/mL (2%) and 721 000 copies/mL, respectively. HCV RNA measured by quantitative PCR was 45 000 000 IU/mL, while the a-fetoprotein level was o5 ng/mL. Other haematological values were not significant and ethanol and urine drug screens were negative.The patient's abdominal complaints did not change during the course of her admission and an abdominal ultrasound revealed no evidence of cirrhosis, focal liver lesions, intrahepatic duct dilatation or hepatic vein thrombosis. The common bile duct was measured and found to be normal. Fungal, mycobacterium,...

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Vanishing bile duct syndrome in Hodgkin's disease: case report

Cited by 22 publications

References 10 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

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

Vanishing bile duct syndrome in a patient with advanced AIDS

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