Allogeneic haematopoietic stem cell transplantation eliminates alloreactive inhibitory antibodies after liver transplantation for bile salt export pump deficiency

Florian Brinkert, Ieva Pukite, Dorothee Krebs-Schmitt, Andrea Briem-Richter, Jan Stindt, Dieter Häussinger, Verena Keitel, Ingo Müller, Enke Grabhorn

Research output: Contribution to journalArticlepeer-review

3 Citations (Scopus)

Abstract

Progressive familial intrahepatic cholestasis 2 is an autosomal-recessive disorder caused by mutations in the ABCB11 gene, which encodes the bile salt export pump (BSEP). Recurrence of BSEP deficiency after liver transplantation is caused by the development of anti-BSEP antibodies. Antibody-induced BSEP deficiency is typically treated by increasing immunosuppressive therapy. We report, in a child, the first case of allogeneic haematopoietic stem cell transplantation for antibody-induced BSEP deficiency that was refractory to intensive pharmacological immunosuppression and immunoadsorption. After haematopoietic stem cell transplantation, anti-BSEP antibodies were cleared from the patient's serum and later from the canalicular space of the liver graft.

Original languageEnglish
Pages (from-to)961-965
Number of pages5
JournalJournal of Hepatology
Volume69
Issue number4
DOIs
Publication statusPublished - Oct 2018
Externally publishedYes

Field of Science

  • 3.2 Clinical medicine

Publication Type

  • 1.1. Scientific article indexed in Web of Science and/or Scopus database

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