Systemic Epstein-Barr virus-positive T-cell lymphoproliferative disease of childhood: Report of a case with review of the literature

Yoshii,Miyuki; Ishida,Mitsuaki; Hodohara,Keiko; Okuno,Hiroko; Nakanishi,Ryota; Yoshida,Takashi; Okabe,Hidetoshi

doi:10.3892/ol.2012.754

September 2012 Volume 4 Issue 3

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September 2012 Volume 4 Issue 3

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Article

Systemic Epstein-Barr virus-positive T-cell lymphoproliferative disease of childhood: Report of a case with review of the literature

Authors:
- Miyuki Yoshii
- Mitsuaki Ishida
- Keiko Hodohara
- Hiroko Okuno
- Ryota Nakanishi
- Takashi Yoshida
- Hidetoshi Okabe
View Affiliations / Copyright

Affiliations: Department of Clinical Laboratory Medicine, Shiga University of Medical Science, Otsu, Shiga 520-2192, Japan, Department of Hematology, Shiga University of Medical Science, Otsu, Shiga 520-2192, Japan
Pages: 381-384
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Published online on: June 12, 2012

https://doi.org/10.3892/ol.2012.754
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Abstract

Systemic Epstein‑Barr virus (EBV)‑positive T‑cell lymphoproliferative disease (LPD) of childhood is an extremely rare and distinct clinicopathological entity. The majority of these cases occur with an apparent primary EBV infection. In this study, we describe a case of systemic EBV‑positive T‑cell LPD of childhood in a 23‑year‑old female with primary EBV infection, and review the clinicopathological features of this disease. A 23‑year‑old previously healthy female without an immunocompromized status presented with an acute onset of high fever. Laboratory examinations revealed a markedly elevated white blood cell count and liver and renal function. Peripheral blood smears identified a number of atypical lymphocytes with small azurophilic granules in the cytoplasm. Bone marrow aspiration revealed marked proliferation of small‑sized lymphocytes with convoluted nuclei, which expressed EBER1, CD3, CD8 and cytotoxic granules. Monoclonal rearrangements of T‑cell receptors were also detected. The patient underwent chemotherapy, but succumbed to multiorgan failure 20 weeks after administration. Upon review of 17 cases of this disease, including the one in the present study, we identified that the major clinicopathological features of systemic EBV-positive T-cell LPD of childhood are as follows: i) clonal systemic proliferation of EBV‑infected T-cells that appear morphologically innocuous with an activated cytotoxic phenotype; ii) a high prevalence in the Asian population, commonly affecting children and young adults; iii) a predilection for males; iv) most commonly involved sites are the liver, spleen, lymph node and bone marrow, and the main clinical presentations are hepatosplenomegaly, fever and pancytopenia; v) almost all cases have an aggressive clinical course, which results in mortality. Cytological atypia of the neoplastic cells in this disease, as observed in the present case, is minimal. This study revealed that the cytomorphological features of atypical lymphocytes in the peripheral blood are indistinguishable from those of infectious mononucleosis.

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Journals

International Journal of Molecular Medicine

International Journal of Oncology

Molecular Medicine Reports

Oncology Reports

Experimental and Therapeutic Medicine

Oncology Letters

Biomedical Reports

Molecular and Clinical Oncology

World Academy of Sciences Journal

International Journal of Functional Nutrition

International Journal of Epigenetics

Medicine International

Systemic Epstein-Barr virus-positive T-cell lymphoproliferative disease of childhood: Report of a case with review of the literature

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