Authors: Koichiro Haruki Hiroaki Shiba Junichi Shimada Norimitsu Okui Tomonori Iida Katsuhiko Yanaga
Publish Date: 2016/10/31
Volume: 10, Issue: 1, Pages: 47-51
Abstract
Posttransplant lymphoproliferative disorder PTLD is a lifethreatening complication of solid organ transplantation We herein report a case of PTLD after livingdonor liver retransplantation reLDLT for recurrent primary sclerosing cholangitis PSC for which complete response was achieved by surgical resection and rituximab A 47yearold man who had undergone livingdonor liver transplantation LDLT twice at age of 43 and 45 years for endstage liver disease firstly for PSC and secondary for recurrent PSC suffered liver dysfunction due to an acute cellular rejection ACR 17 months after reLDLT At reLDLT a right liver lobe was donated from his spouse Although steroid was effective for ACR PTLD developed in the ileocecal area The patient received rituximab for treatment of PTLD and ileocecal resection for hemorrhage from ileocecal PTLD The patient achieved complete response by rituximab and surgical resection for PTLD but PSC recurred and hemophagocytic syndrome HPS developed with hyperbilirubinemia and elevated serum ferritin The patient received steroid treatment for HPS but thrombocytopenia and coagulopathy developed presumably due to thrombotic microangiopathy Therefore tacrolimus was switched to mycophenolate mofetil Despite intensive treatment including plasmapheresis and platelet infusion fungal infection of both lungs developed and the patient died 22 months after reLDLT Autopsy revealed complete response of PTLD recurrence of PSC and persistance of HPS
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