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

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.

journal_name

J Hepatol

journal_title

Journal of hepatology

authors

Brinkert F,Pukite I,Krebs-Schmitt D,Briem-Richter A,Stindt J,Häussinger D,Keitel V,Müller I,Grabhorn E

doi

10.1016/j.jhep.2018.06.003

subject

Has Abstract

pub_date

2018-10-01 00:00:00

pages

961-965

issue

4

eissn

0168-8278

issn

1600-0641

pii

S0168-8278(18)32156-1

journal_volume

69

pub_type

杂志文章

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