Vanishing bile duct syndrome in a hodgkin′s lymphoma patient with fatal outcome despite lymphoma remission

Abstract

Vanishing bile duct syndrome (VBDS) is a condition resulting from severe bile duct injury, progressive destruction, and disappearance of intrahepatic bile ducts (ductopenia) leading to cholestasis, biliary cirrhosis, and liver failure. VBDS can be associated with a variety of disorders, including Hodgkin's lymphoma (HL). We describe a 33-year-old male patient who presented with lymphadenopathy and jaundice, and was diagnosed to have HL. Serum bilirubin worsened progressively despite chemotherapy, with a cholestatic pattern of liver enzymes. Diagnosis of VBDS was established on liver biopsy. Although remission from HL was achieved, the patient died of liver failure. Presence of jaundice in HL patients should raise the possibility of VBDS. This report discusses the difficulties of delivering chemotherapy in patients with liver dysfunction. HL-associated VBDS carries a high mortality but lymphoma remission can be achieved in some patients. Therefore, liver transplantation should be considered early in these patients.

Fatal vanishing bile duct syndrome in Iranian patient with Hodgkin's lymphoma

“…Notably, this high mortality rate is mostly related to liver dysfunction rather than lymphoma progression. This clarifies the difficulties encountered in the administration of potential hepatotoxic chemotherapy in severely cholestatic patients 8,9 …”

“…The suitable management of HL-related VBDS remains controversial, as both conventional chemotherapy and alternative regimens have been reported to be successful in achieving remission in this condition. 9,[12][13][14] Additionally, radiotherapy could increase liver failure-free survival, while chemoradiation is a treatment option that in many patients finally causes remarkable liver failure, needing liver transplantation. 12,15 The available literature has reported a high mortality rate in patients with HL-related VBDS despite adequate treatment.…”

Paraneoplastic Intrahepatic Cholestasis in Supradiaphragmatic Classical Hodgkin Lymphoma Successfully Treated With Brentuximab Vedotin: A Case Report and Review of the Literature

“…In other cases, nitrogen mustards (9,15,22), mainly mechlorethamine, have been used in jaundiced cHL patients. ESHAP (etoposide, methylprednisolone, cytarabine, cisplatin) in modified drug doses has also been administered in a case report (23), leading to only a mild decrease of total bilirubin. Rituximab (8,20) has been combined with BEACOPP-14 (20) and gemcitabine/cisplatin (8) leading to bilirubin normalization after several months.…”

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

“…[ 38 ] 1 Yes Full No Cure Umit H. [ 39 ] 1 Unknown Unknown Unknown Unknown Gill R.M. [ 40 ] 1 Yes Reduced No Hepatic failure Foramiti S. [ 41 ] 1 Yes Full Yes Hepatic failure Gagnon MF [ 42 ] 1 Yes Full No Cure Wong KM [ 43 ] 1 Yes Full No Cure Aleem A1 [ 44 ] 1 Yes Full No Hepatic failure Nader K [ 45 ] 1 Yes Full No Hepatic failure Our patient 1 Yes Full Yes Cure *either chemotherapy or radiotherapy. …”