Skip to main content

Advertisement

SpringerLink
Log in

EBV-PTLD in a patient after haploidentical stem-cell transplantation with post-transplant cyclophosphamide

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

Abstract

Here, we describe the case of a male patient with Epstein-Barr virus post-transplantation lymphoproliferative disorder (EBV-PTLD), which developed 18 months after a haploidentical hematopoietic stem cell transplantation (haplo-HSCT) and the administration of post-transplant cyclophosphamide (PTCy). Of note, no anti-thymoglobulin was used in the entire clinical course. Prior to the onset of EBV-PTLD, the patient had pulmonary chronic graft-versus-host disease and was treated with prednisolone and tacrolimus. After stopping immunosuppressive therapy, he was diagnosed with EBV-positive infectious mononucleosis PTLD, and EBV-associated hemophagocytic syndrome; therefore, dexamethasone and rituximab monotherapies were administered. After four courses of rituximab, EBV-DNA was no longer detected in the peripheral blood, and the patient’s laboratory data improved. Overall, this study highlights the need to predict the risk factors associated with the development of EBV-PTLD in transplanted patients after haplo-HSCT with PTCy.

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

Similar content being viewed by others

References

  1. Liu L, Zhang X, Feng S. Epstein-Barr Virus-related post-transplantation lymphoproliferative disorders after allogeneic hematopoietic stem cell transplantation. Biol Blood Marrow Transplant. 2018;24:1341–9.

    Article  Google Scholar 

  2. Styczynski J, Gil L, Tridello G, Ljungman P, Donnelly JP, van der Velden W, et al. Response to rituximab-based therapy and risk factor analysis in Epstein Barr Virus-related lymphoproliferative disorder after hematopoietic stem cell transplant in children and adults: a study from the Infectious Diseases Working Party of the European Group for Blood and Marrow Transplantation. Clin Infect Dis. 2013;57:794–802.

    Article  CAS  Google Scholar 

  3. Uhlin M, Wikell H, Sundin M, Blennow O, Maeurer M, Ringden O, et al. Risk factors for Epstein-Barr virus-related post-transplant lymphoproliferative disease after allogeneic hematopoietic stem cell transplantation. Haematologica. 2014;99:346–52.

    Article  Google Scholar 

  4. Styczynski J, van der Velden W, Fox CP, Engelhard D, de la Camara R, Cordonnier C, et al. Management of Epstein-Barr Virus infections and post-transplant lymphoproliferative disorders in patients after allogeneic hematopoietic stem cell transplantation: Sixth European Conference on Infections in Leukemia (ECIL-6) guidelines. Haematologica. 2016;101:803–11.

    Article  CAS  Google Scholar 

  5. Gagelmann N, Bacigalupo A, Rambaldi A, Hoelzer D, Halter J, Sanz J, et al. Haploidentical stem cell transplantation with posttransplant cyclophosphamide therapy vs other donor transplantations in adults with hematologic cancers: a systematic review and meta-analysis. JAMA Oncol. 2019;5:1739–48.

    Article  Google Scholar 

  6. Clay J, Kulasekararaj AG, Potter V, Grimaldi F, McLornan D, Raj K, et al. Nonmyeloablative peripheral blood haploidentical stem cell transplantation for refractory severe aplastic anemia. Biol Blood Marrow Transplant. 2014;20:1711–6.

    Article  Google Scholar 

  7. Mohyuddin GR, Roller J, Shune L, Lin T, Dias A, Ganguly S, et al. Epstein-Barr viremia and post-transplant lymphoproliferative disorders in patients undergoing haploidentical stem cell transplantation with post-transplant cyclophosphamide. Hematol Oncol Stem Cell Ther. 2019;12:171–3.

    Article  Google Scholar 

  8. Kanakry JA, Kasamon YL, Bolaños-Meade J, Borrello IM, Brodsky RA, Fuchs EJ, et al. Absence of post-transplantation lymphoproliferative disorder after allogeneic blood or marrow transplantation using post-transplantation cyclophosphamide as graft-versus-host disease prophylaxis. Biol Blood Marrow Transplant. 2013;19:1514–7.

    Article  Google Scholar 

  9. Al Hamed R, Bazarbachi AH, Mohty M. Epstein-Barr virus-related post-transplant lymphoproliferative disease (EBV-PTLD) in the setting of allogeneic stem cell transplantation: a comprehensive review from pathogenesis to forthcoming treatment modalities. Bone Marrow Transplant. 2020;55:25–39.

    Article  CAS  Google Scholar 

  10. Hickey CL, Romee R, Nikiforow S, Dorfman D, Mazzeo M, Koreth J. A case of Epstein Barr virus-related post-transplant lymphoproliferative disorder after haploidentical allogeneic stem cell transplantation using post-transplantation cyclophosphamide. Haematologica. 2020;105:e379–81.

    Article  Google Scholar 

  11. 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;127:2375–90.

    Article  CAS  Google Scholar 

  12. Salas MQ, Prem S, Remberger M, Lam W, Kim DDW, Michelis FV, et al. High incidence but low mortality of EBV-reactivation and PTLD after alloHCT using ATG and PTCy for GVHD prophylaxis. Leuk Lymphoma. 2020;61:3198–208.

    Article  CAS  Google Scholar 

  13. Raiola AM, Dominietto A, Ghiso A, Grazia CD, Lamparelli T, Gualandi F, et al. Unmanipulated haploidentical bone marrow transplantation and posttransplantation cyclophosphamide for hematologic malignancies after myeloablative conditioning. Biol Blood Marrow Transplant. 2013;19:117–22.

    Article  CAS  Google Scholar 

  14. Raiola AM, Dominietto A, di Grazia C, Lamparelli T, Gualandi F, Ibatici A, et al. Unmanipulated haploidentical transplants compared with other alternative donors and matched sibling grafts. Biol Blood Marrow Transplant. 2014;20:1573–9.

    Article  Google Scholar 

  15. Liu Q, Xuan L, Liu H, Huang F, Zhou H, Fan Z, et al. Molecular monitoring and stepwise preemptive thrapy for Epstein-Barr virus viremia after allogeneic stem cell transplantation. Am J Hematol. 2013;88:550–5.

    Article  CAS  Google Scholar 

  16. Landgren O, Gilbert ES, Rizzo JD, Socié G, Banks PM, Sobocinski KA, et al. Risk factors for lymphoproliferative disorders after allogeneic hematopoietic cell transplantation. Blood. 2009;113:4992–5001.

    Article  CAS  Google Scholar 

  17. Rasche L, Kapp M, Einsele H, Mielke S. EBV-induced post-transplant lymphoproliferative disorders: a persisting challenge in allogeneic hematopoetic SCT. Bone Marrow Transplant. 2014;49:163–7.

    Article  CAS  Google Scholar 

Download references

Acknowledgements

We thank Editage (http://www.editage.jp) for English language editing. No funding was received for this study.

Author information

Authors and Affiliations

  1. Division of Hematology/Oncology, Department of Internal Medicine, Kameda Medical Center, 929 Higashi-chou, Kamogawa, Chiba, 296-8602, Japan

    Toshiki Terao, Takafumi Tsushima, Ami Fukumoto, Ayumi Kuzume, Daisuke Miura, Kentaro Narita, Masami Takeuchi & Kosei Matsue

Authors
  1. Toshiki Terao
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Takafumi Tsushima
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Ami Fukumoto
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Ayumi Kuzume
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Daisuke Miura
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Kentaro Narita
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Masami Takeuchi
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Kosei Matsue
    View author publications

    You can also search for this author in PubMed Google Scholar

Contributions

TT*: wrote and edited the manuscript and performed patient care and the literature review; TT, AF, AK, DM, KN, and MT: provided patient care; KM: supervised the study and provided patient care. All authors reviewed and approved the final version of the manuscript.

Corresponding author

Correspondence to Toshiki Terao.

Ethics declarations

Conflict of interest

The authors declare that they have no conflicts of interest.

Consent

The patient and family provided consent for the publication of this case with the removal of all identifying information to remain anonymous and retain their privacy.

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

Terao, T., Tsushima, T., Fukumoto, A. et al. EBV-PTLD in a patient after haploidentical stem-cell transplantation with post-transplant cyclophosphamide. Int J Hematol 114, 136–140 (2021). https://doi.org/10.1007/s12185-021-03111-z

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s12185-021-03111-z

Keywords

Search

Navigation