Plasmapharesis, Intravenous Immunoglobulin and Rituximab Successfully Treat Recurrent Progressive Familial Intrahepatic Cholestasis Type 2 (PFIC-2) After Liver Transplantation
1Liver Trasplantation, King Faisal Specialist Hospital & Research Center, Riyadh, Saudi Arabia
2Gastroenterology, King Saud University, Riyadh, Saudi Arabia
3Pathology, King Faisal Specialist Hospital & Research Center, Riyadh, Saudi Arabia.
Meeting: 2015 American Transplant Congress
Abstract number: A211
Keywords: Immunoglobulins (Ig), Immunosuppression, Liver transplantation
Session Information
Session Name: Poster Session A: Liver: Immunosuppression and Rejection
Session Type: Poster Session
Date: Saturday, May 2, 2015
Session Time: 5:30pm-7:30pm
Presentation Time: 5:30pm-7:30pm
Location: Exhibit Hall E
Background:
Allo-immune mediated BSEP dysfunction may occur after liver transplantation in PFIC2 patients leading to a PFIC2 like phenotype. The IgG antibodies are reactive toward a canalicular epitope of BSEP, are of high affinity, and inhibited transport activity of BSEP, thus causing severe cholestasis. This phenomenon was first described in 2009, since then, few cases of PFIC-2 recurrence were reported with mixed results.
Methods:
We report on two patients who developed recurrent normal GGT cholestasis mimicking primary BSEP disease, after liver transplantation. A 14 years old boy and his 19 years old sister who had received cadaveric liver transplantation at the United States in 2011. In January 2014 they presented with severe itching, high bilirubin, high AST/ALT, high serum bile acid with persistently low GGT. Virology, Autoimmune screen, Abdominal CT Scan, ERCP and liver biopsy were negative. Immunosuppressions were maximized with no improvement. A repeat biopsy of the 14 year-old boy on May 2014 showed recurrence of PFIC2, His Anti-BSEP came positive with a very high serum titer 1:1200, Treatment regimen for him started on June 2014, he received a course of 5 sessions of plasmapharesis each session followed by IV immunoglobulin (IVIG) , then received first dose of I.V. Rituximab 375/m2. The second course of Plasmapharesis where modified by doing 5 sessions of plasmapharesis every other day with an exchange volume of 1.5, followed by 3 days of IVIG to avoid washing out the IVIG by plasmapharesis, followed by the second dose of IV Rituximab 375/m2. His sister's liver biopsy on July 2014 showed PFIC2 recurrence. She started treatment for recurrence started on September 2014, using the modified protocol. She is receiving the second course of Plasmapharesis, IVIG and IV Rituximab now.
Results:
Currently, both patients improved clinically and biochemically and still on treatment plan.
Conclusions:
PFIC-2 recurrence after liver transplantation occur through an antibody mediated reaction against BSEP receptors on canalicular membrane and can successfully be treated with plasmapharesis, IVIG and rituximab obviating the need for re-transplantation
To cite this abstract in AMA style:
Elsiesy H, Abaalkhail F, Alhamoudi W, Alhussaini H, Alsebayel M, Broring D, Shagrani M. Plasmapharesis, Intravenous Immunoglobulin and Rituximab Successfully Treat Recurrent Progressive Familial Intrahepatic Cholestasis Type 2 (PFIC-2) After Liver Transplantation [abstract]. Am J Transplant. 2015; 15 (suppl 3). https://atcmeetingabstracts.com/abstract/plasmapharesis-intravenous-immunoglobulin-and-rituximab-successfully-treat-recurrent-progressive-familial-intrahepatic-cholestasis-type-2-pfic-2-after-liver-transplantation/. Accessed May 12, 2025.« Back to 2015 American Transplant Congress