TY - JOUR
T1 - Allogeneic haematopoietic stem cell transplantation eliminates alloreactive inhibitory antibodies after liver transplantation for bile salt export pump deficiency
AU - Brinkert, Florian
AU - Pukite, Ieva
AU - Krebs-Schmitt, Dorothee
AU - Briem-Richter, Andrea
AU - Stindt, Jan
AU - Häussinger, Dieter
AU - Keitel, Verena
AU - Müller, Ingo
AU - Grabhorn, Enke
N1 - Funding Information:
VK received funding from the Deutsche Forschungsgemeinschaft (DFG) for the Klinische Forschergruppe 217 ( KFO 217 ).
Publisher Copyright:
© 2018 European Association for the Study of the Liver
PY - 2018/10
Y1 - 2018/10
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=85051244183&partnerID=8YFLogxK
U2 - 10.1016/j.jhep.2018.06.003
DO - 10.1016/j.jhep.2018.06.003
M3 - Article
C2 - 29935200
AN - SCOPUS:85051244183
SN - 0168-8278
VL - 69
SP - 961
EP - 965
JO - Journal of Hepatology
JF - Journal of Hepatology
IS - 4
ER -