Clinical Journal of Gastroenterology

, Volume 10, Issue 1, pp 47–51 | Cite as

Complete response to post-transplant lymphoproliferative disorder by surgical resection and rituximab after living-donor liver re-transplantation for recurrent primary sclerosing cholangitis

  • Koichiro HarukiEmail author
  • Hiroaki Shiba
  • Junichi Shimada
  • Norimitsu Okui
  • Tomonori Iida
  • Katsuhiko Yanaga
Case Report


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.


Post-transplant lymphoproliferative disorder Primary sclerosing cholangitis Liver transplantation Surgical resection 


Compliance with ethical standards

Conflict of interest:

The authors declare that they have no conflict of interest.

Human/Animal Rights:

All procedures followed were in accordance with the ethical standards of the responsible committee on human experimentation (institutional and national) and with the Helsinki Declaration of 1975, as revised in 2008(5).

Informed Consent:

Informed consent was obtained from all patients to be included in the study.


  1. 1.
    Opelz G, Döhler B. Lymphomas after solid organ transplantation: a collaborative transplant study report. Am J Transpl. 2004;4:222–30.CrossRefGoogle Scholar
  2. 2.
    Muti G, Cantoni S, Oreste P, Cooperative Study Group on PTLDs, et al. Post-transplant lymphoproliferative disorders: improved outcome after clinico-pathologically tailored treatment. Haematologica. 2002;87:67–77.PubMedGoogle Scholar
  3. 3.
    Jain A, Nalesnik M, Reyes J, et al. Posttransplant lymphoproliferative disorders in liver transplantation: a 20-year experience. Ann Surg. 2002;236:429–37.CrossRefPubMedPubMedCentralGoogle Scholar
  4. 4.
    Swerdlow SH, Webber SA, Chadburn A. Post-transplant lymphoproliferative disorders. WHO classification of tumours of haematopoietic and lymphoid tissues. Lyon: IARC; 2008.Google Scholar
  5. 5.
    Ghobrial IM, Habermann TM, Maurer MJ, et al. Prognostic analysis for survival in adult solid organ transplant recipients with post-transplantation lymphoproliferative disorders. J Clin Oncol. 2005;23:7574–82.CrossRefPubMedGoogle Scholar
  6. 6.
    Mendizabal M, Marciano S, dos Santos Schraiber L, et al. Post-transplant lymphoproliferative disorder in adult liver transplant recipients: a South American multicenter experience. Clin Transpl. 2013;27:E469–77.CrossRefGoogle Scholar
  7. 7.
    Reshef R, Vardhanabhuti S, Luskin MR, et al. Reduction of immunosuppression as initial therapy for posttransplantation lymphoproliferative disorder. Am J Transpl. 2011;11:336–47.CrossRefGoogle Scholar
  8. 8.
    Choquet S, Leblond V, Herbrecht R, et al. Efficacy and safety of rituximab in B-cell post-transplantation lymphoproliferative disorders: results of a prospective multicenter phase 2 study. Blood. 2006;107:3053–7.CrossRefPubMedGoogle Scholar
  9. 9.
    Cruz RJ Jr, Ramachandra S, Sasatomi E, et al. Surgical management of gastrointestinal posttransplant lymphoproliferative disorders in liver transplant recipients. Transplantation. 2012;94:417–23.CrossRefPubMedGoogle Scholar
  10. 10.
    Smith JM, Corey L, Healey PJ, et al. Adolescents are more likely to develop posttransplant lymphoproliferative disorder after primary Epstein-Barr virus infection than younger renal transplant recipients. Transplantation. 2007;83:1423–8.CrossRefPubMedGoogle Scholar
  11. 11.
    Lim WH, Russ GR, Coates PT. Review of Epstein-Barr virus and post-transplant lymphoproliferative disorder post-solid organ transplantation. Nephrology (Carlton). 2006;11:355–66.CrossRefGoogle Scholar
  12. 12.
    Petrara MR, Giunco S, Serraino D, et al. Post-transplant lymphoproliferative disorders: from epidemiology to pathogenesis-driven treatment. Cancer Lett. 2015;369:37–44.CrossRefPubMedGoogle Scholar
  13. 13.
    Campo E, Swerdlow SH, Harris NL, et al. The 2008 WHO classification of lymphoid neoplasms and beyond: evolving concepts and practical applications. Blood. 2011;117:5019–32.CrossRefPubMedPubMedCentralGoogle Scholar
  14. 14.
    Hauke R, Smir B, Greiner T, et al. Clinical and pathological features of posttransplant lymphoproliferative disorders: influence on survival and response to treatment. Ann Oncol. 2001;12:831–4.CrossRefPubMedGoogle Scholar
  15. 15.
    Heslop HE. How I treat EBV lymphoproliferation. Blood. 2009;114:4002–8.CrossRefPubMedPubMedCentralGoogle Scholar
  16. 16.
    Graziadei IW, Wiesner RH, Batts KP, et al. Recurrence of primary sclerosing cholangitis following liver transplantation. Hepatology. 1999;29:1050–6.CrossRefPubMedGoogle Scholar
  17. 17.
    Alexander J, Lord JD, Yeh MM, et al. Risk factors for recurrence of primary sclerosing cholangitis after liver transplantation. Liver Transpl. 2008;14:245–51.CrossRefPubMedGoogle Scholar
  18. 18.
    Egawa H, Ueda Y, Ichida T, et al. Risk factors for recurrence of primary sclerosing cholangitis after living donor liver transplantation in Japanese registry. Am J Transpl. 2011;11:518–27.CrossRefGoogle Scholar
  19. 19.
    Ishii E, Ohga S, Imashuku S, et al. Nationwide survey of hemophagocytic lymphohistiocytosis in Japan. Int J Hematol. 2007;86:58–65.CrossRefPubMedGoogle Scholar
  20. 20.
    Soyama A, Eguchi S, Takatsuki M, et al. Hemophagocytic syndrome after liver transplantation: report of two cases. Surg Today. 2011;41:1524–30.CrossRefPubMedGoogle Scholar
  21. 21.
    Shindoh J, Sugawara Y, Akamatsu N, et al. Thrombotic microangiopathy after living-donor liver transplantation. Am J Transpl. 2012;12:728–36.CrossRefGoogle Scholar
  22. 22.
    Hori T, Kaido T, Oike F, et al. Thrombotic microangiopathy-like disorder after living-donor liver transplantation: a single-center experience in Japan. World J Gastroenterol. 2011;17:1848–57.CrossRefPubMedPubMedCentralGoogle Scholar

Copyright information

© Japanese Society of Gastroenterology 2016

Authors and Affiliations

  1. 1.Department of SurgeryThe Jikei University School of MedicineTokyoJapan

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