title: Systematic Epstein-Barr virus-positive T-cell lymphoproliferative disease presenting as a persistent fever and cough: a case report.
abstract: BACKGROUND
  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.
  
  
  METHODS
  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.
authors.forename: Fereshteh
authors.initials: F
authors.lastname: Ameli
authors.forename: Firouzeh
authors.initials: F
authors.lastname: Ghafourian
authors.affiliation: Department of Pathology, Faculty of Medicine, Universiti Kebangsaan Malaysia Medical Centre, Jalan Yaacob Latif, Bandar Tun Razak 56000, Cheras, Kuala Lumpur, Malaysia. noraidah@ppukm.ukm.edu.my.
authors.forename: Noraidah
authors.initials: N
authors.lastname: Masir
mesh_terms: D000293:Adolescent; D001706:Biopsy; D003371:Cough / Q000209:etiology* / Q000821:virology; D003937:Diagnosis, Differential; D020031:Epstein-Barr Virus Infections / Q000150:complications* / Q000175:diagnosis* / Q000821:virology; D017809:Fatal Outcome; D005334:Fever / Q000209:etiology* / Q000821:virology; D006801:Humans; D008232:Lymphoproliferative Disorders / Q000150:complications* / Q000175:diagnosis* / Q000821:virology; D008297:Male; D009102:Multiple Organ Failure / Q000209:etiology / Q000821:virology; D010198:Pancytopenia / Q000209:etiology / Q000821:virology; D018805:Sepsis / Q000209:etiology / Q000821:virology; D013601:T-Lymphocytes / Q000821:virology