Vanishing bile duct syndrome and Hodgkin disease: a case series and review of the literature

J Pediatr Hematol Oncol. 2008 Dec;30(12):976-80. doi: 10.1097/MPH.0b013e31818b37c4.

Abstract

This is the first description in which the diagnosis of vanishing bile duct syndrome (VBDS) preceded the diagnosis of Hodgkin disease (HD) by several months, and for which patients received modifications to modern MOPP-ABV chemotherapy with successful clinical remission. VBDS is an uncommon form of liver disease manifested by severe cholestasis and progressive liver failure. We report 2 cases of stage IIIB pediatric HD and VBDS. Because VBDS is progressive and the only curative treatment is liver transplant, it is imperative to recognize that children with VBDS may also have concurrent HD.

Publication types

  • Case Reports

MeSH terms

  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use*
  • Bleomycin / therapeutic use
  • Child
  • Cholestasis / diagnosis*
  • Cholestasis / drug therapy
  • Combined Modality Therapy
  • Doxorubicin / therapeutic use
  • Hodgkin Disease / diagnosis*
  • Hodgkin Disease / drug therapy
  • Humans
  • Male
  • Mechlorethamine / therapeutic use
  • Prednisone / therapeutic use
  • Procarbazine / therapeutic use
  • Radiotherapy Dosage
  • Remission Induction
  • Syndrome
  • Treatment Outcome
  • Vinblastine / therapeutic use
  • Vincristine / therapeutic use

Substances

  • Bleomycin
  • Procarbazine
  • Mechlorethamine
  • Vincristine
  • Vinblastine
  • Doxorubicin
  • Prednisone

Supplementary concepts

  • MOPP-ABV protocol