Colestasis idiopática asociada a ductopenia progresiva en dos pacientes con linfoma de Hodgkin

Ripoll, Cristina; Bañares, Rafael; Carretero, Lola; Sabin, P; Álvarez, E.; Marrupe, D.

doi:10.1016/s0210-5705(02)79026-2

Cited by 16 publications

(4 citation statements)

References 17 publications

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“…A less well known but particularly serious problem is the development of vanishing bile duct syndrome (VBDS). Including our patient, 18 cases with this complication have been reported [11][12][13][14]. As in our patient, most cases presented with neck swelling later on jaundice.…”

Section: Discussionmentioning

confidence: 50%

Severe Jaundice, due to Vanishing Bile Duct Syndrome in Hodgkin's Lymphoma, Fully Reversible after Chemotherapy

2023

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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 and imaging study the absence of lymphoma cells in liver. We describe a 13-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: 50%

Severe Jaundice, due to Vanishing Bile Duct Syndrome in Hodgkin's Lymphoma, Fully Reversible after Chemotherapy

2023

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“…[3]1YesFullYesCureOzkan A. [26]1NoNoLateHepatic failureRipoll C. [27]2YesFullNoHepatic failureYesFullYesCureKomurcu S. [28]1YesFullNoHepatic failureLiangpunsakul S. [29]1YesFullNoCureGuliter S. [30]1YesFullLateHepatic failureCórdoba Iturriagagoitia A. [31]1YesFullNoCureHan W.S.…”

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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“…As Hubscher et al (24) described, ductopenia may also develop in Hodgkin's disease. On occasion, the patient may even present with liver-related symptoms (25), and thus it should be considered for differential diagnosis. The patient here had no infectious symptoms, which are common in cell-mediated immunodeficiency; no adenopathies or organomegalies were identified upon palpation.…”

Section: Dr Gonzalo Gómezmentioning

confidence: 99%

“…(24), la ductopenia puede desarrollarse también en la enfermedad de Hodgkin. En ocasiones incluso el paciente debuta con clínica hepática (25), por lo que resulta importante incluirla en el diagnóstico diferencial. La paciente que discutimos no presentó clínica infecciosa propia de la inmunodeficiencia celular, a la exploración no se palparon adenopatías ni se halló hepatoo esplenomegalia.…”

Section: Dr Gonzalo Gómezunclassified

A young woman with cholestasis and ductopenia

Gómez

Gil

Muñoz

et al. 2004

Rev. esp. enferm. dig.

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and development of mostly incomplete septa with only a few complete porto-portal fibrous bridges. Spatial relationships were adequately preserved between portal tracts and centroacinar veins. No interlobular ducts were seen in over 50% of portal spaces (6 of 11), with presence of hepatic arteries (Fig. 2). Furthermore, portal spaces had a mild mixed inflammatory infiltrate with predominant lymphocytes. The few remaining interlobular ducts had an irregular biliary epithelium. Within the limiting periportal trabecule foci of collagen-entrapped hepatocytes were seen. Periportal hepatocytes were clearly changed and had copper deposits (Figs. 3 and 4). In the remaining lobule, liver cells had their typical characteristics or showed mild hyperplastic features. No positive globules were seen using the periodic acid-Schiff (PAS) stain. No viral inclusions were apparent. Bile pig

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Colestasis idiopática asociada a ductopenia progresiva en dos pacientes con linfoma de Hodgkin

Cited by 16 publications

References 17 publications

Severe Jaundice, due to Vanishing Bile Duct Syndrome in Hodgkin's Lymphoma, Fully Reversible after Chemotherapy

Severe Jaundice, due to Vanishing Bile Duct Syndrome in Hodgkin's 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

A young woman with cholestasis and ductopenia

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