Case Report
Copyright ©The Author(s) 2015. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Hepatol. Sep 8, 2015; 7(19): 2229-2236
Published online Sep 8, 2015. doi: 10.4254/wjh.v7.i19.2229
Liver transplantation in a patient with primary antiphospholipid syndrome and Budd-Chiari syndrome
Tatiana M Reshetnyak, Natalia V Seredavkina, Maria A Satybaldyeva, Evgeniy L Nasonov, Vasiliy I Reshetnyak
Tatiana M Reshetnyak, Natalia V Seredavkina, Maria A Satybaldyeva, Evgeniy L Nasonov, VA Nasonova Research Institute of Rheumatology, Kashirskoye Shosse, Moscow 115522, Russian Federation
Tatiana M Reshetnyak, Russian Medical Academy of Postgraduate Education, Moscow 125993, Russian Federation
Vasiliy I Reshetnyak, VA Negorovsky Research Institute of General Reanimatology, Moscow 107031, Russian Federation
Author contributions: All authors contributed to the acquisition of data, writing, and revision of this manuscript.
Supported by VA Nasonova Scientific Research Institute of Rheumatology, Moscow, Russian Federation.
Institutional review board statement: This case report was exempt from the Institutional Review Board standards at VA Nasonova Scientific Research Institute of Rheumatology, Russian Medical Academy of Postgraduate Education and VA Negorovsky Research Institute of General Reanimatology in Moscow, Russian Federation.
Institutional review board statement: The patient involved in this study gave his written informed consent authorizing use and disclosure of his protected health information.
Conflict-of-interest statement: All the authors have no conflicts of interests to declare.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Vasiliy I Reshetnyak, MD, PhD, DSc of Medicine, Professor, Academic Secretary, VA Negovsky Research Institute of General Reanimatology, 25-2, Petrovka Str, Moscow 107031, Russian Federation. vasiliy.reshetnyak@yandex.ru
Telephone: +7-495-6946505 Fax: +7-495-6946505
Received: February 24, 2015
Peer-review started: February 26, 2015
First decision: June 18, 2015
Revised: August 10, 2015
Accepted: August 20, 2015
Article in press: August 21, 2015
Published online: September 8, 2015
Abstract

The antiphospholipid syndrome (APS) is an acquired thrombophilic disorder in which autoantibodies are produced to a variety of phospholipids determinants of cell membranes or phospholipid binding proteins. There are few reports about association between antiphospholipid antibodies and development of Budd-Chiari syndrome (BCS). We report the case of BCS development in young Russian male with primary APS. The patient underwent orthotopic liver transplantation on August 26, 2012. At present time his state is good, the blood flow in the liver restored and its function is not impaired. We report about the first time the successful use of dabigatran etexilate for prolonged anticoagulation therapy in APS patient with BCS. In addition patient is managed with immunosuppressive drugs.

Keywords: Budd-Chiari syndrome, Antiphospholipid syndrome, Inherent thrombophilia, Antiphosphlipid antibodies, Orthotopic liver transplantation

Core tip: Budd-Chiari syndrome (BCS) is rare disease with a potentially dismal outcome if not treated optimally. In manuscript is reported the case report of the BCS development in young Russian male with primary antiphospholipid syndrome (APS), who was underwent orthotopic liver transplantation and now is managed with immunosupressive drugs and with prolonged anticoagulation. For the first time, it is reported the successful use of dabigatran etexilate for prolongation anticoagulation therapy in primary APS patient with BCS.