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Successful treatment with rituximab and donor lymphocyte infusions for fulminant EBV-associated lymphoproliferative disorder that developed 14 years after unrelated BMT

Bone Marrow Transplantation volume 46, pages 1583–1585 (2011)Cite this article

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Epstein-Barr virus (EBV)-associated lymphoproliferative disorder (EBV-LPD) results from the outgrowth of EBV-infected B cells that would normally be controlled by an effective EBV-specific cytotoxic T-cell (CTL) response.1 EBV-LPD occurs after allo-SCT as an EBV-associated post transplantation lymphoproliferative disorder (EBV-PTLD), with an overall incidence of ∼1% and a mortality rate as high as 50–90%.2, 3, 4 Almost all cases of EBV-PTLD develop within the first year of transplantation before reconstitution of EBV-specific CTL response; however, it occurs later in the most severely immunocompromised patients, and extensive chronic GVHD is the only risk factor for late-onset EBV-PTLD.2 Recently, the survival of patients with EBV-PTLD has been improved by combination chemotherapy with rituximab and donor lymphocyte infusions (DLI).5, 6 Fulminant EBV-LPD is another type of fatal EBV-associated disease, which is caused by the proliferation of EBV-infected T cells. Here, we report an unusual case of fulminant EBV-LPD with meningoencephalitis that developed 14 years after unrelated BMT. Furthermore, EBV-infected B and T cells were successfully eliminated by rituximab and DLI.

The present case has unusual characteristics and features that are different from typical cases of EBV-PTLD in many ways: disease onset, symptoms, severity, immune status and the type of proliferative EBV-infected cells. The patient had developed EBV-LPD 14 years after unrelated BMT; he had been free of chronic GVHD and immunosuppressive drugs for most of this time. The typical pattern of EBV-infected B-cell proliferation was not seen, but the case showed EBV-infected T-cell as well as B-cell proliferation. Quintanilla-Martinez et al. reported six cases of fulminant T-cell EBV-LPD that progressed to multiple organ failure, sepsis and death after initial or chronic infection of EBV.8 The symptoms and pathogenesis of the patient were more consistent with fulminant T-cell EBV-LPD than typical EBV-PTLD, although there was no report of fulminant EBV-LPD following transplantation.

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Authors and Affiliations

  1. Department of Hematology, Chiba University Hospital, Chiba, Japan

    T Kawaguchi, S Tsukamoto, C Ohwada, M Takeuchi, T Muto, S Tanaka, S Sakai, Y Takeda, D Abe, E Sakaida, N Shimizu, T Iseki & C Nakaseko

  2. Department of Blood Transfusion, Chiba University Hospital, Chiba, Japan

    T Kawaguchi, T Muto, N Shimizu & T Iseki

  3. Department of Clinical Cell Biology and Medicine, Chiba University Graduate School of Medicine, Chiba, Japan

    K Yokote

  4. Department of Infectious Diseases, National Research Institute for Child Health and Development, Tokyo, Japan

    K-I Imadome

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  1. T Kawaguchi
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Correspondence to C Nakaseko.

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Kawaguchi, T., Tsukamoto, S., Ohwada, C. et al. Successful treatment with rituximab and donor lymphocyte infusions for fulminant EBV-associated lymphoproliferative disorder that developed 14 years after unrelated BMT. Bone Marrow Transplant 46, 1583–1585 (2011). https://doi.org/10.1038/bmt.2010.346

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