Hodgkin lymphoma-related vanishing bile duct syndrome and idiopathic cholestasis: Statistical analysis of all published cases and literature review

Ballonoff, Ari; Kavanagh, Brian D.; Nash, Russell; Drabkin, Harry A.; Trotter, James; Costa, Luciano J.; Rabinovitch, Rachel

doi:10.1080/02841860701644078

Cited by 65 publications

(83 citation statements)

References 25 publications

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“…Outcomes in cases of VBDS and IC range from complete resolution of liver abnormalities with appropriate therapy to permanent hepatic failure and death [5]. There have been multiple case reports of HL-related VBDS and IC, all of lymph node origin [5]. We report a case of primary GI HL with evidence of liver involvement, but also with prominent ductopenia on liver biopsy and associated intrahepatic cholestasis.…”

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

confidence: 97%

“…Clinical signs of VBDS include intrahepatic cholestasis and evidence of loss of bile ducts (i.e., ductopenia) on biopsy, but without direct involvement of the liver by lymphoma [7]. Outcomes in cases of VBDS and IC range from complete resolution of liver abnormalities with appropriate therapy to permanent hepatic failure and death [5]. There have been multiple case reports of HL-related VBDS and IC, all of lymph node origin [5].…”

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

confidence: 99%

“…An equally uncommon clinical presentation of HL is that of cholestatic liver failure, which may be the result of intrahepatic lymphomatous involvement, extrahepatic compression from mass lesions, or toxic reactions to medications or ionizing radiation. A rare paraneoplastic syndrome previously described in association with HL presenting with cholestatic liver failure is the vanishing bile duct syndrome (VBDS), a subtype of idiopathic cholestasis (IC) [4][5][6]. Clinical signs of VBDS include intrahepatic cholestasis and evidence of loss of bile ducts (i.e., ductopenia) on biopsy, but without direct involvement of the liver by lymphoma [7].…”

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

confidence: 99%

“…The classic definition of VBDS excludes patients with liver involvement by HL, as VBDS is thought to be due to a paraneoplastic process rather than direct involvement by HL [5]. Patients with cholestatic liver failure related to direct liver involvement by HL may have some focal portal tract obliteration related to the inflammatory lymphomatous infiltrate but do not demonstrate frank ductopenia [4][5][6].…”

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

confidence: 99%

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

confidence: 97%

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

confidence: 99%

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

confidence: 99%

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

confidence: 99%

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

et al. 2011

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“…The authors concluded that idiopathic cholestasis and VBDS due to HL are potentially reversible entities, with a good liver and lymphoma outcomes noticed in 30% of patients. But the restoration of liver function depends on obtaining complete response to HL therapy, which should be done as soon as possible [59].…”

Section: Hodgkin's Lymphoma and Vanishing Bile Duct Syndromementioning

confidence: 99%

Liver Involvement in Hodgkin's Lymphoma: Types of Injuries and Therapeutic Implications

Mihăilă

2017

IJPPE

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Abstract. The hepatocytolysis raises questions on following therapeutic conduct when it occurs during chemotherapy for Hodgkin's lymphoma, expression of its liver toxicity. But the onset of primary liver Hodgkin's lymphoma, including the form manifested by acute liver failure, poses even greater problems, as in the case of occurrence of vanishing bile duct syndrome -expression of a paraneoplastic syndrome, hemophagocytic lymphohistiocytosis, peliosis hepatis or association of lymphoma with infection with hepatitis viruses or human immunodeficiency virus or different autoimmune diseases. This review summarizes the clinical experience acquired on the relationship between Hodgkin's lymphoma and liver, from the point of view of clinical manifestations, used treatments and clinical evolution. Suggestions on the course of treatment in patients with Hodgkin's lymphoma and liver damage have been formulated starting from the metabolism and elimination of chemotherapy drugs and taking into account the clinical experience of published clinical trials and cases. This review is a synthesis of knowledge obtained in this field, during the time, of therapeutic possibilities and limits, and formulates potential future milestones for research.

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Lymphadenopathy and Jaundice

Law

Gopal

2015

JAMA Oncol

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A Hispanic man in his 20s with no known medical history presented with worsening abdominal pain over 5 days, persistent pruritus, and scleral icterus. The patient noted swelling on the right side of the neck and a 10-lb weight loss over the past 2 months. He reported a history of moderate alcohol consumption, drinking 1 beer per day during the week and approximately 5 to 8 beers over the weekend, but denied any use of nutritional supplements or illicit drugs. There was no recent travel outside the state. Human immunodeficiency virus test results on admission were negative. Physical examination revealed scleral icterus, jaundice, and a fixed right supraclavicular lymph node conglomerate measuring 6 × 3 cm. There was no cervical, left supraclavicular, axillary, or inguinal lymphadenopathy. Computed tomography of the neck, chest, abdomen, and pelvis demonstrated a large right supraclavicular lymph node, extensive mediastinal lymphadenopathy, a large extracardiac mass abutting the left atrium, hepatomegaly, and multiple splenic lesions. Laboratory evaluation demonstrated a total serum bilirubin level of 10.5 mg/dL with a direct component of 8.4 mg/dL (to convert to micromoles per liter, multiply by 17.104). Renal function was normal. Mild leukopenia and slight thrombocytopenia in the absence of associated anemia was noted on initial complete blood cell count. A liver biopsy was performed, with results shown in Figure 1.

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Hodgkin lymphoma-related vanishing bile duct syndrome and idiopathic cholestasis: Statistical analysis of all published cases and literature review

Cited by 65 publications

References 25 publications

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

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

Liver Involvement in Hodgkin's Lymphoma: Types of Injuries and Therapeutic Implications

Lymphadenopathy and Jaundice

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