Skip to main content

Advertisement

SpringerLink
Log in

NK-cell post-transplant lymphoproliferative disease successfully treated by second allogenic hematopoietic stem cell transplantation in chronic active Epstein–Barr virus infection

  • Case Report
  • Published:
International Journal of Hematology Aims and scope Submit manuscript

Abstract

Chronic active Epstein–Barr virus infection (CAEBV) is a systemic T- or NK-lymphoproliferative disorder (LPD) caused by EBV. Allogenic hematopoietic stem cell transplantation (HSCT) is the only curative therapy for CAEBV, but relapse sometimes occurs. Relapse is generally attributed to proliferation of recipient-derived CAEBV cells. We herein report a case of donor-derived CAEBV-like NK-cell post-transplant lymphoproliferative disease (PTLD) in a 41-year-old female after the first allogenic HSCT for CAEBV from an HLA-matched sibling donor. A second HSCT from an HLA-matched unrelated donor successfully controlled the disease, but EBV infection of cells derived from the second donor continued to be detected. Although the mechanisms underlying CAEBV and CAEBV-like NK-cell PTLD have not yet been elucidated in detail, the findings of the present case imply that host genetic factors, including familial factors, may be important in disease development.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3

Similar content being viewed by others

References

  1. Epstein MA, Achong BG, Barr YM. Virus particle in cultured lymphocytes from Burkitt’s lymphoma. Lancet. 1964;1(7335):702–3.

    Article  CAS  Google Scholar 

  2. Babcock GJ, Decker LL, Volk M, Thorley-Lawson DA. EBV persistence in memory B cells in vivo. Immunity. 1998;9(3):395–404.

    Article  CAS  Google Scholar 

  3. Virelizier JL, Lenoir G, Griscelli C. Persistent Epstein-Barr virus infection in a child with hypergammaglobulinaemia and immunoblastic proliferation associated with a selective defect in immune interferon secretion. Lancet. 1978;2(8083):231–4.

    Article  CAS  Google Scholar 

  4. Swerdlow SH, Campo E, Pileri SA, Harris NL, Stein H, Siebert R, et al. The 2016 revision of the World Health Organization classification of lymphoid neoplasms. Blood. 2016;127(20):2375–90.

    Article  CAS  Google Scholar 

  5. Yonese I, Sakashita C, Imadome KI, Kobayashi T, Yamamoto M, Sawada A, et al. Nationwide survey of systemic chronic active EBV infection in Japan in accordance with the new WHO classification. Blood Adv. 2020;4(13):2918–26.

    Article  CAS  Google Scholar 

  6. Sawada A, Inoue M, Kawa K. How we treat chronic active Epstein-Barr virus infection. Int J Hematol. 2017;105(4):406–18.

    Article  CAS  Google Scholar 

  7. Cohen JI, Jaffe ES, Dale JK, Pittaluga S, Heslop HE, Rooney CM, et al. Characterization and treatment of chronic active Epstein-Barr virus disease: a 28-year experience in the United States. Blood. 2011;117(22):5835–49.

    Article  CAS  Google Scholar 

  8. Shi M, Nguyen P, Timm MM, Otteson GE, Horna P, Olteanu H, et al. Cytoplasmic expression of CD3ε heterodimers by flow cytometry rapidly distinguishes between mature T-cell and natural killer-cell neoplasms. Am J Clin Pathol. 2020;154(5):683–91.

    Article  CAS  Google Scholar 

  9. Arai A, Imadome K, Wang L, Wu N, Kurosu T, Wake A, et al. Recurrence of chronic active Epstein-Barr virus infection from donor cells after achieving complete response through allogeneic bone marrow transplantation. Intern Med. 2012;51(7):777–82.

    Article  Google Scholar 

  10. Yui S, Yamaguchi H, Imadome K, Arai A, Takahashi M, Ohashi R, et al. Epstein-Barr Virus-positive T-cell lymphoproliferative disease following umbilical cord blood transplantation for acute myeloid leukemia. J Nippon Med Sch. 2016;83(1):35–42.

    Article  CAS  Google Scholar 

  11. Iemura T, Kondo T, Hishizawa M, Yamashita K, Kimura H, Takaori-Kondo A. NK-cell post-transplant lymphoproliferative disease with chronic active Epstein-Barr virus infection-like clinical findings. Int J Infect Dis. 2019;88:31–3.

    Article  CAS  Google Scholar 

  12. Sugaya N, Kimura H, Hara S, Hoshino Y, Kojima S, Morishima T, et al. Quantitative analysis of Epstein-Barr virus (EBV)-specific CD8+ T cells in patients with chronic active EBV infection. J Infect Dis. 2004;190(5):985–8.

    Article  Google Scholar 

  13. Shibayama H, Imadome KI, Onozawa E, Tsuzura A, Miura O, Koyama T, et al. Virus-specific cytotoxic T cells in chronic active Epstein-Barr virus infection. Rinsho Ketsueki. 2017;58(6):583–8.

    PubMed  Google Scholar 

  14. Sawada A, Inoue M. Hematopoietic stem cell transplantation for the treatment of Epstein-Barr virus-associated T- or NK-cell lymphoproliferative diseases and associated disorders. Front Pediatr. 2018;6:334.

    Article  Google Scholar 

  15. Okuno Y, Murata T, Sato Y, Muramatsu H, Ito Y, Watanabe T, et al. Defective Epstein-Barr virus in chronic active infection and haematological malignancy. Nat Microbiol. 2019;4(3):404–13.

    Article  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Hematology, Kitano hospital, Medical Research Institute, 2-4-20, Ougimachi, Kita-ku, Osaka, Japan

    Sho Shibata, Yoko Takiuchi, Naoto Kawasaki, Yoshio Okamoto, Shojiro Inano, Akiko Fukunaga, Sumie Tabata & Toshiyuki Kitano

  2. Division of Hematology and Oncology, Department of Internal Medicine, St. Marianna University School of Medicine, Kawasaki, Japan

    Ayako Arai

  3. Department of Hematological Therapeutics, Tokyo Medical and Dental University (TMDU), Tokyo, Japan

    Ayako Arai

  4. Department of Advanced Medicine for Virus Infections, National Center for Child Health and Development, Tokyo, Japan

    Ken-Ichi Imadome

Authors
  1. Sho Shibata
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Yoko Takiuchi
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Naoto Kawasaki
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Yoshio Okamoto
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Shojiro Inano
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Akiko Fukunaga
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Sumie Tabata
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Ayako Arai
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Ken-Ichi Imadome
    View author publications

    You can also search for this author in PubMed Google Scholar

  10. Toshiyuki Kitano
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Yoko Takiuchi.

Ethics declarations

Conflict of interest

The authors state that they have no Conflicts of Interest (COIs).

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Shibata, S., Takiuchi, Y., Kawasaki, N. et al. NK-cell post-transplant lymphoproliferative disease successfully treated by second allogenic hematopoietic stem cell transplantation in chronic active Epstein–Barr virus infection. Int J Hematol 115, 595–599 (2022). https://doi.org/10.1007/s12185-021-03271-y

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s12185-021-03271-y

Keywords

Search

Navigation