International Journal of Hematology

, Volume 108, Issue 1, pp 112–117 | Cite as

Systemic Epstein–Barr virus-positive T-cell lymphoproliferative disorders of childhood with fulminant leukocytosis and tumor lysis: a case report with autopsy findings

  • Sachie Wada
  • Takayuki Suzuki
  • Koichi Kitazume
  • Akira Fujita
  • Seiichiro Shimizu
Case Report


Systemic Epstein–Barr virus (EBV)-positive T-cell lymphoproliferative disorders (T-LPD) of childhood is an extremely rare disease characterized by an aggressive clinical course and very poor prognosis. We report an adolescent male with systemic EBV-positive T-LPD of childhood after primary EBV infection, resulting in a fatal clinical course within 9 days, along with autopsy findings. A 19-year-old male without an immunocompromised status presented with an acute onset of high fever, and was hospitalized for persistent fever, vomiting and diarrhea on the 5th day from onset. Laboratory data showed severe thrombocytopenia, increased ferritin level, liver dysfunction, disseminated intravascular coagulation, and anti-EBV-IgM positivity. Peripheral blood smears identified a number of atypical lymphocytes. Bone marrow aspiration revealed many atypical various-sized lymphocytes with apparent nucleoli and hemophagocytosis. Atypical lymphocytes displayed a CD8+ T-cell phenotype with monoclonal rearrangement of T-cell receptors. EBV-encoded RNA was also observed in lymphoid cells by in situ hybridization. The patient received dexamethasone and cyclosporine with no improvement, and died of tumor lysis by leukocytosis on the 9th day from onset.


Epstein–Barr virus (EBV) T-cell lymphoproliferative disorders (T-LPD) Fulminant Leukocytosis Tumor lysis 



This case of diagnosis was supported by the Department of Pathology at Kurume University School of Medicine. We thank Koichi Ohshima and Kensuke Kawamoto for their assistance.

Compliance with ethical standards

Conflict of interest

All authors declare no conflict of interest.


  1. 1.
    Quintanilla-Martinez L, Kimura H, Jaffe E. EBV-positive T-cell lymphoproliferative disorders of childfood. In: Swerdlow SH, Campo E, Harris NL, et al., editors. WHO classification of tumours of haematopoietic and lymphoid tissues. 4th ed. Lyon: International Agency for Research on Cancer Press; 2008. p. 278–80.Google Scholar
  2. 2.
    Kanegane H, Kanegane C, Yachie A, Miyawaki T, Tosato G. Infectious mononucleosis as a disease of early childhood in Japan caused by primary Epstein–Barr virus infection. Acta Paediatr Jpn. 1997;39:166–71.CrossRefPubMedGoogle Scholar
  3. 3.
    Yoshii M, Ishida M, Hodohara K, Okuno H, Nakanishi R, Yoshida T, et al. Systemic Epstein–Barr virus-positive T-cell lymphoproliferative disease of childhood: report of a case with review of the literature. Oncol Lett. 2012;4:381–4.CrossRefPubMedPubMedCentralGoogle Scholar
  4. 4.
    Sawada A, Inoue M, Kawa K. How we treat chronic active Epstein–Barr virus infection. Int J Hematol. 2017;105:406–18.CrossRefPubMedGoogle Scholar
  5. 5.
    Quintanilla-Martinez L, Kumer S, Fend F, Reyes E, Teruya-Feldstein J, Kingma DW, et al. Fulminant EBV(+) T-cell lymphoproliferative disorder following acute/chronic EBV infection: a distinct clinicopathologic syndrome. Blood. 2000;96:443–51.PubMedGoogle Scholar
  6. 6.
    Chen G, Chen L, Qin X, Huang Z, Xie X, Li G, et al. Systemic Epstein–Barr virus positive T-cell lymphoproliferative disease of childhood with hemophagocytic syndrome. Int J Clin Exp Pathol. 2014;7:7110–3.PubMedPubMedCentralGoogle Scholar
  7. 7.
    Ohshima K, Kimura H, Yoshino T, Kim CW, Ko YH, Lee SS, et al. Proposed categorization of pathological states of EBV-associated T/natural killer-cell lymphoproliferative disorder (LPD) in children and young adults: overlap with chronic active EBV infection and infantile fulminant EBV T-LPD. Pathol Int. 2008;58:209–17.CrossRefPubMedGoogle Scholar
  8. 8.
    Kimura H, Ito Y, Kawabe S, Gotoh K, Takahashi Y, Kojima S, et al. EBV-associated T/NK-cell lymphoproliferative diseases in nonimmunocompromised hosts: prospective analysis of 108 cases. Blood. 2012;119:673–86.CrossRefPubMedGoogle Scholar
  9. 9.
    Suzuki R, Nakamura S, Suzumiya J, Ichimura K, Ichikawa M, Ogata K, et al. Blastic natural killer cell lymphoma/leukemia (CD56-positive blastic tumor): prognostication and categorization according to anatomic sites of involvement. Cancer. 2005;104:1022–31.CrossRefPubMedGoogle Scholar

Copyright information

© The Japanese Society of Hematology 2017

Authors and Affiliations

  1. 1.Department of HematologyShowa General HospitalTokyoJapan
  2. 2.Department of PathologyShowa General HospitalTokyoJapan

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