title: Systemic Epstein-Barr-virus-positive T cell lymphoproliferative childhood disease in a 22-year-old Caucasian man: A case report and review of the literature.
abstract: BACKGROUND
  Systemic Epstein-Barr-virus-positive T cell lymphoproliferative disease of childhood is an extremely rare disorder, characterized by clonal proliferation of Epstein-Barr-virus-infected T cells with an activated cytotoxic phenotype. The disease is more frequent in Asia and South America, with only few cases reported in Western countries. A prompt diagnosis, though often difficult, is a necessity due to the very aggressive clinical course of the disease.
  
  
  METHODS
  We report the clinicopathological features of fulminant T cell lymphoproliferative disease that arose in the setting of acute primary Epstein-Barr virus infection. Our patient, a 23-year-old man, presented to our facility with persisting fever, hepatosplenomegaly and severe pancytopenia. On bone marrow biopsy, an abundant lymphoid infiltrate was observed. Immunophenotypic and molecular studies revealed that the atypical lymphoid cells displayed a CD8+, Epstein-Barr-encoded-RNA-positive T cell phenotype with clonal rearrangement of the T cell receptor genes, the final diagnosis being systemic Epstein-Barr-virus-positive T cell lymphoproliferative disease. On reviewing the literature we found only 14 similar cases, all presenting with very aggressive clinical courses and requiring extensive phenotyping and molecular techniques for final diagnosis.
  
  
  CONCLUSIONS
  Though extremely rare, this disease can occur in Europe, and a comprehensive diagnostic approach is thus recommended in all case of Epstein-Barr-virus-positive lymphoproliferative disorders. Unfortunately, at present no specific treatment is available; however, prompt administration of anti- Epstein-Barr virus treatment and rapid attempts to control the hemophagocytic syndrome are indicated.
authors.affiliation: Department of Hematology and Oncological Sciences 'L and A Seràgnoli', Hematopathology Section, S Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy. pierpaolo.piccaluga@unibo.it.
authors.forename: Valentina
authors.initials: V
authors.lastname: Tabanelli
authors.forename: Claudio
authors.initials: C
authors.lastname: Agostinelli
authors.forename: Elena
authors.initials: E
authors.lastname: Sabattini
authors.forename: Anna
authors.initials: A
authors.lastname: Gazzola
authors.forename: Francesco
authors.initials: F
authors.lastname: Bacci
authors.forename: Saveria
authors.initials: S
authors.lastname: Capria
authors.forename: Claudia
authors.initials: C
authors.lastname: Mannu
authors.forename: Simona
authors.initials: S
authors.lastname: Righi
authors.forename: Maria Teresa
authors.initials: MT
authors.lastname: Sista
authors.forename: Giovanna
authors.initials: G
authors.lastname: Meloni
authors.forename: Stefano A
authors.initials: SA
authors.lastname: Pileri
authors.forename: Pier Paolo
authors.initials: PP
authors.lastname: Piccaluga