title: Complete response to post-transplant lymphoproliferative disorder by surgical resection and rituximab after living-donor liver re-transplantation for recurrent primary sclerosing cholangitis.
abstract: Post-transplant lymphoproliferative disorder (PTLD) is a life-threatening complication of solid organ transplantation. We herein report a case of PTLD after living-donor liver re-transplantation (reLDLT) for recurrent primary sclerosing cholangitis (PSC), for which complete response was achieved by surgical resection and rituximab. A 47-year-old man, who had undergone living-donor liver transplantation (LDLT) twice at age of 43 and 45 years for end-stage 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.
authors: Department of Surgery, The Jikei University School of Medicine, 3-25-8, Nishi-Shinbashi, Minato-ku, Tokyo, 105-8461, Japan. haruki@jikei.ac.jp.
authors: Koichiro
authors: http://orcid.org/0000-0002-1686-3228
authors: K
authors: Haruki
authors: Department of Surgery, The Jikei University School of Medicine, 3-25-8, Nishi-Shinbashi, Minato-ku, Tokyo, 105-8461, Japan.
authors: Hiroaki
authors: H
authors: Shiba
authors: Department of Surgery, The Jikei University School of Medicine, 3-25-8, Nishi-Shinbashi, Minato-ku, Tokyo, 105-8461, Japan.
authors: Junichi
authors: J
authors: Shimada
authors: Department of Surgery, The Jikei University School of Medicine, 3-25-8, Nishi-Shinbashi, Minato-ku, Tokyo, 105-8461, Japan.
authors: Norimitsu
authors: N
authors: Okui
authors: Department of Surgery, The Jikei University School of Medicine, 3-25-8, Nishi-Shinbashi, Minato-ku, Tokyo, 105-8461, Japan.
authors: Tomonori
authors: T
authors: Iida
authors: Department of Surgery, The Jikei University School of Medicine, 3-25-8, Nishi-Shinbashi, Minato-ku, Tokyo, 105-8461, Japan.
authors: Katsuhiko
authors: K
authors: Yanaga
mesh_terms: D002760:Cholangiopancreatography, Endoscopic Retrograde; D015209:Cholangitis, Sclerosing / Q000601:surgery*; D003113:Colonoscopy; D003131:Combined Modality Therapy; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use*; D016031:Liver Transplantation / Q000009:adverse effects*; D019520:Living Donors; D008232:Lymphoproliferative Disorders / Q000000981:diagnostic imaging / Q000209:etiology / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D012008:Recurrence; D012086:Reoperation / Q000379:methods; D000069283:Rituximab / Q000627:therapeutic use*; D014057:Tomography, X-Ray Computed
chemical_list: D007155:Immunologic Factors; D000069283:Rituximab
keywords: Liver transplantation; Post-transplant lymphoproliferative disorder; Primary sclerosing cholangitis; Surgical resection