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Abstract: TH-PO1043

A Case of Epstein-Barr Virus-Associated Extranodal NK/T-Cell Lymphoma Developing 16 Years After Kidney Transplantation

Session Information

Category: Transplantation

  • 2002 Transplantation: Clinical

Authors

  • Shimizu, Sakura, Kanazawa Daigaku Fuzoku Byoin, Kanazawa, Ishikawa, Japan
  • Yamaguchi, Kaori, Toyama Kenritsu Chuo Byoin, Toyama, Toyama, Japan
  • Funamoto, Tomoaki, Toyama Kenritsu Chuo Byoin, Toyama, Toyama, Japan
  • Shinozaki, Yasuyuki, Toyama Kenritsu Chuo Byoin, Toyama, Toyama, Japan
  • Yoshida, Misaki, Kanazawa Daigaku Fuzoku Byoin, Kanazawa, Ishikawa, Japan
  • Koshino, Akihiko, Kanazawa Daigaku Fuzoku Byoin, Kanazawa, Ishikawa, Japan
  • Sako, Keisuke, Kanazawa Daigaku Fuzoku Byoin, Kanazawa, Ishikawa, Japan
  • Yuasa, Takahiro, Kanazawa Daigaku Fuzoku Byoin, Kanazawa, Ishikawa, Japan
  • Minami, Taichiro, Kanazawa Daigaku Fuzoku Byoin, Kanazawa, Ishikawa, Japan
  • Nishioka, Ryo, Kanazawa Daigaku Fuzoku Byoin, Kanazawa, Ishikawa, Japan
  • Oshima, Megumi, Kanazawa Daigaku Fuzoku Byoin, Kanazawa, Ishikawa, Japan
  • Yamamura, Yuta, Kanazawa Daigaku Fuzoku Byoin, Kanazawa, Ishikawa, Japan
  • Hara, Satoshi, Kanazawa Daigaku Fuzoku Byoin, Kanazawa, Ishikawa, Japan
  • Ito, Kiyoaki, Kanazawa Daigaku Fuzoku Byoin, Kanazawa, Ishikawa, Japan
  • Mizushima, Ichiro, Kanazawa Daigaku Fuzoku Byoin, Kanazawa, Ishikawa, Japan
  • Sakai, Norihiko, Kanazawa Daigaku Fuzoku Byoin, Kanazawa, Ishikawa, Japan
  • Iwata, Yasunori, Kanazawa Daigaku Fuzoku Byoin, Kanazawa, Ishikawa, Japan
Introduction

Post-transplant lymphoproliferative disorder (PTLD) is a serious complication following organ transplantation, and approximately 90% of cases are associated with Epstein–Barr virus (EBV) infection. Most PTLDs are of B-cell origin and typically arise in the transplanted organ or the central nervous system. We report a rare case of late-onset EBV-associated extranodal NK/T-cell lymphoma (ENKTL) after kidney transplantation.

Case Description

A 70-year-old man underwent living-donor kidney transplantation 16 years ago for end-stage kidney disease secondary to chronic glomerulonephritis. He had received tacrolimus, methylprednisolone, and mizoribine. He was referred to our hospital because of recurrent right nasal obstruction persisting for two years. Systemic CT revealed lesions extending from the right nasal cavity to the paranasal sinuses without lymphadenopathy. Histopathological examination demonstrated mixed infiltration of CD20+ B cells and CD3+ T cells with a small number of CD56+ cells. Increased numbers of EBV-encoded small RNA (EBER) + cells suggested EBV-associated PTLD. Peripheral blood examination showed an elevated EBV-DNA copy number. Reduction of immunosuppressive agents was attempted; however, EBV positivity and increased soluble interleukin-2 receptor levels persisted, and rituximab therapy was initiated. Although EBV-DNA subsequently became undetectable, the patient developed recurrent nasal obstruction, and FDG-PET demonstrated progression of the sinonasal lesions. Repeat biopsy revealed atypical CD56+ lymphoid infiltration with increased EBER+ cells, leading to a diagnosis of ENKTL. The lesion was localized to the nasal cavity without distant metastasis. Radiotherapy combined with DeVIC chemotherapy achieved complete remission.

Discussion

NK/T-cell PTLD is uncommon, and nasal involvement has been infrequently reported. In the present case, EBV-associated ENKTL developed 16 years after kidney transplantation. Although the lesion was initially suspected to represent B-cell PTLD, disease progression occurred despite rituximab therapy, and repeat biopsy established the diagnosis of ENKTL. Late-onset PTLD may exhibit more diverse and complex histopathological features compared with early-onset PTLD. Repeated pathological evaluation should be considered in refractory or progressive lesions after transplantation.