International Journal of Hematology

, Volume 109, Issue 2, pp 221–227 | Cite as

Epstein–Barr virus-related diffuse large B-cell lymphoma in mogamulizumab-treated adult T-cell leukemia with incomplete T-cell reconstitution

  • Kazuharu Kamachi
  • Takero ShindoEmail author
  • Masaharu Miyahara
  • Kazutaka Kitaura
  • Michiaki Akashi
  • Tadasu Shin-I
  • Ryuji Suzuki
  • Koichi Oshima
  • Shinya Kimura
Case Report


Adult T-cell leukemia (ATL) is an aggressive mature T-cell malignancy with a poor prognosis. The anti-C–C motif chemokine receptor 4 (CCR4) antibody mogamulizumab (moga) reduces ATL cells and induces reconstitution of polyclonal T cells; however, ATL cases often remain resistant and moga sometimes causes fatal immunopathology. Epstein–Barr virus (EBV)-related B-cell lymphoma develops in severely immunocompromised subjects, and is particularly associated with impaired T-cell immunity. Here, we report an ATL patient who had received conventional chemotherapy plus moga, and subsequently developed EBV-related diffuse large B-cell lymphoma (DLBCL) of the central nervous system. Next-generation sequencing-based T-cell receptor repertoire analyses identified residual abnormal clones and revealed that reconstitution of polyclonal T cells was incomplete, even after moga treatment. Furthermore, a skin rash that developed after moga treatment was found to contain ATL clones. This case suggests that the limited therapeutic effects of moga and incomplete T-cell reconstitution are associated with severely impaired T-cell immunity and subsequent development of EBV-related DLBCL.


Adult T-cell leukemia (ATL/ATLL) Epstein–Barr virus-related central nervous system lymphoma Mogamulizumab T-cell reconstitution 




Compliance with ethical standards

Conflict of interest

The author(s) declare that they have no conflict of interest.


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Copyright information

© The Japanese Society of Hematology 2018

Authors and Affiliations

  1. 1.Department of Internal MedicineKaratsu Red Cross HospitalKaratsuJapan
  2. 2.Division of Hematology, Respiratory Medicine and Oncology, Department of Internal Medicine, Faculty of MedicineSaga UniversitySagaJapan
  3. 3.Department of Hematology/OncologyKyoto University Graduate School of MedicineKyotoJapan
  4. 4.Repertoire Genesis IncIbarakiJapan
  5. 5.Department of PathologyKaratsu Red Cross HospitalKaratsuJapan
  6. 6.BITS Co. LtdTokyoJapan
  7. 7.Department of Clinical Immunology, Clinical Research Center for Allergy and RheumatologySagamihara National HospitalSagamiharaJapan
  8. 8.Department of PathologyKurume University School of MedicineKurumeJapan

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