Systematic Epstein-Barr virus-positive T-cell lymphoproliferative disease presenting as a persistent fever and cough: A case report

Fereshteh Ameli, Firouzeh Ghafourian, Noraidah Masir

Research output: Contribution to journalArticle

4 Citations (Scopus)

Abstract

Introduction. Systemic Epstein-Barr virus-positive T-cell lymphoproliferative childhood disease is an extremely rare disorder and classically arises following primary acute or chronic active Epstein-Barr virus infection. It is characterized by clonal proliferation of Epstein-Barr virus-infected T-cells with an activated cytotoxic phenotype. This disease has a rapid clinical course and is more frequent in Asia and South America, with relatively few cases being reported in Western countries. The clinical and pathological features of the disease overlap with other conditions including infectious mononucleosis, chronic active Epstein-Barr virus infection, hemophagocytic lymphohistiocytosis and natural killer cell malignancies. We describe the rare case of systemic Epstein-Barr virus-positive T-cell lymphoproliferative childhood disease in a 16-year-old Malay boy.

Case presentation. He presented with a six-month history of fever and cough, with pulmonary and mediastinal lymphadenopathy and severe pancytopenia. Medium- to large-sized, CD8+ and Epstein-Barr virus-encoded RNA-positive atypical lymphoid cells were present in the bone marrow aspirate. He subsequently developed fatal virus-associated hemophagocytic syndrome and died due to sepsis and multiorgan failure.

Conclusions: Although systemic Epstein-Barr virus-positive T-cell lymphoproliferative childhood disease is a disorder which is rarely encountered in clinical practice, our case report underlines the importance of a comprehensive diagnostic approach in the management of this disease. A high level of awareness of the disease throughout the diagnosis process for young patients who present with systemic illness and hemophagocytic syndrome may be of great help for the clinical diagnosis of this disease.

Original languageEnglish
Article number288
JournalJournal of Medical Case Reports
Volume8
Issue number1
DOIs
Publication statusPublished - 27 Aug 2014

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Human Herpesvirus 4
Cough
Fever
T-Lymphocytes
Hemophagocytic Lymphohistiocytosis
Epstein-Barr Virus Infections
Infectious Mononucleosis
Pancytopenia
South America
Disease Management
Natural Killer Cells
Sepsis
Bone Marrow
Lymphocytes
RNA
Viruses
Phenotype
Lung
Neoplasms

Keywords

  • Childhood
  • Epstein-Barr virus
  • T-cell lymphoproliferative disease

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Systematic Epstein-Barr virus-positive T-cell lymphoproliferative disease presenting as a persistent fever and cough : A case report. / Ameli, Fereshteh; Ghafourian, Firouzeh; Masir, Noraidah.

In: Journal of Medical Case Reports, Vol. 8, No. 1, 288, 27.08.2014.

Research output: Contribution to journalArticle

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