Vanishing bile duct syndrome related to DILI and Hodgkin lymphoma overlap: A rare and severe case

Greca, Raquel Dias; Cunha‐Silva, Marlone; Costa, Larissa Bastos Eloy da; Costa, Júlia Guimarães Fernandes; Mazo, Daniel Ferraz de Campos; Sevá‐Pereira, Tiago; Nascimento, Marlla M.C.; Pereira, Isadora E.; Oliveira, Flávia Carvalho Bitencourt de; Faria, Guilherme Amorim Souza; Neto, Fernando Lopes Ponte; Almeida, José R.

doi:10.1016/j.aohep.2019.06.010

Cited by 20 publications

(8 citation statements)

References 21 publications

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“…The mechanism of VBDS has been described in the literature to be due to variety of hepatic insults resulting in progressive destruction and disappearance of intrahepatic bile ducts and ultimately leading to cholestasis The association between VBDS and Hodgkin lymphoma was first explained in 1993 in three patients with intrahepatic cholestasis and ductopenia by Hubscher et al [4]. The proposed pathophysiology to explain Hodgkin lymphoma-related VBDS is an immune-mediated insult of the biliary epithelium from the autoantibodies produced by the lymphoma or T-cell-mediated toxicity and direct damage to the bile ducts by cytokines [2,5]. Due to paucity in the understanding of the pathophysiology of VBDS, there is not a diagnostic marker or a test, and the syndrome remains a diagnosis of exclusion.…”

Section: Discussionmentioning

confidence: 99%

Vanishing Bile Duct Syndrome in the Presence of Hodgkin Lymphoma

Gaudel¹,

Brown²,

Byrd³

2022

Cureus

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Vanishing bile duct syndrome (VBDS) is an acquired condition characterized by the destruction and loss of intrahepatic bile ducts resulting in cholestasis. VBDS has been described in various conditions including neoplastic and immunologic disorders, infections, hepatic ischemia, and drug toxicity. The diagnosis is confirmed by liver biopsy revealing the loss of interlobular bile ducts in greater than 50% of portal tracts. Prognosis is variable and often unpredictable but appears to be influenced by the etiology of bile duct destruction and overall patient health. VBDS has been described as a rare paraneoplastic process in patients with Hodgkin lymphoma. This case describes a 26-year-old female who presented with a neck mass, jaundice, and pruritus. Initial workup revealed direct hyperbilirubinemia, transaminitis, elevated alkaline phosphatase, and elevated international normalized ratio. She went on to receive a diagnosis of stage II classical Hodgkin lymphoma, nodular sclerosing subtype, and biopsy-proven VBDS. Over the course of chemotherapy, complete metabolic resolution of Hodgkin lymphoma and complete normalization of bilirubin were achieved. She was given gemcitabine and cyclophosphamide as a liver sparing regimen initially with some improvement in liver function tests and a reduction in lymph node volumes. She received six cycles of adriamycin/bleomycin/vinblastine/dacarbazine (ABVD) with complete remission attained after four cycles by positron emission tomography/computed tomography criteria. This report illustrates a safe chemotherapy regimen in the presence of marked liver dysfunction. Workup for VBDS including liver biopsy should be pursued in Hodgkin lymphoma patients with evidence of cholestasis in the absence of extrahepatic bile duct damage or other known etiology of liver injury.

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Section: Discussionmentioning

confidence: 99%

Vanishing Bile Duct Syndrome in the Presence of Hodgkin Lymphoma

Gaudel¹,

Brown²,

Byrd³

2022

Cureus

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“…A patogênese não foi totalmente elucidada. Múltiplos mecanismos já foram propostos, dentre eles: uma resposta imune principalmente mediada por células T, gerando reconhecimento de antígeno na camada de células do epitélio biliar resultando numa infiltração de células imunes na camada intraepitelial dos ductos biliares, apoptose e citotoxicidade por células T (GRECA et al 2020). Hashim et al (2020) descrevem que as anormalidades laboratoriais refletem colestase com elevações da fosfatase alcalina (geralmente maior que três vezes o limite superior do normal), bilirrubina total e gama-glutamil transpeptidase.…”

Section: Discussionunclassified

PESQUISAS EM SAÚDE: RELATOS, EXPERIÊNCIAS E PERSPECTIVAS (2.ª edição)

2022

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A segunda edição do livro "PESQUISAS EM SAÚDE: RELA-TOS, EXPERIÊNCIAS E PERSPECTIVAS" foi organizado com o objetivo de reunir e apresentar resultados de pesquisas e interesses de profissionais e estudantes da saúde e áreas afins de vários lugares do Brasil. Encontramos artigos de revisão, prospecções, estudos de casos e pesquisas experimentais de grande relevância para a literatura científica.Agrademos a Editora Inovar e às equipes envolvidas nesse projeto pelo empenho e confiança.Desejamos a todos uma excelente leitura.

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“…De acordo com Greca et al (2020) as drogas que foram associadas como causa de VBDS incluem: amoxicilina-clavulanato, outras penicilinas, antibióticos macrolídeos, fluoroquinolonas, sulfonamidas, agentes antifúngicos, agentes anti-inflamatórios não esteróides, fenotiazinas, antidepressivos tricíclicos e anticonvulsivantes.…”

Section: Discussionunclassified