title: [Systemic EBV+ T-cell lymphoproliferative disease of childhood].
abstract: Systemic EBV+ T-cell lymphoproliferative disease of childhood is a recent entity described in the 2008 World Health Organisation tumours of haematopoietic system and lymphoid tissues as a clonal T-cell EBV+ systemic proliferation. It occurs after acute or chronic active EBV infection. We report the case of a caucasian, immunocompetent 12-year-old girl, with no particular history, who presented with hemophagocytic lymphohistiocytosis in the aftermath of an infectious mononucleosis. Main symptoms were multiple organ failure, hepatosplenomegaly and pancytopenia. Histopathology of peripheral lymph node and bone marrow revealed a T-cell, CD8+, EBV+ lymphoproliferation. An elevated viral load was detected in blood by PCR. The patient died within 3 weeks. Since most of the cases have been reported in Asia and South America, few cases still have been described in Europe. Unlike B-cell lymphoproliferation in immunocompromised individuals, T-cell EBV+ lymphoproliferation occurs in immunocompetent patients and seems to be the consequence of a proliferative disorder of EBV-infected T-cells, attributed to a cytotoxic T-cell response deficiency. These T-cell proliferations are more frequently immunoreactive for CD8 than CD4. A key feature of the diagnosis might be EBV viral load.
authors.affiliation: Institut de pathologie, CHRU de Lille, 2, avenue Oscar-Lambret, 59037 Lille cedex, France. Electronic address: as.delplanque@chru-lille.fr.
authors.forename: Anne-Sophie
authors.initials: AS
authors.lastname: Lemaire
authors.affiliation: Institut de pathologie, CHRU de Lille, 2, avenue Oscar-Lambret, 59037 Lille cedex, France.
authors.forename: Dorothée
authors.initials: D
authors.lastname: Daussay
authors.affiliation: Institut de pathologie, CHRU de Lille, 2, avenue Oscar-Lambret, 59037 Lille cedex, France.
authors.forename: Brigitte
authors.initials: B
authors.lastname: Bouchindhomme
authors.affiliation: Laboratoire d'hématologie, centre de biologie-pathologie, CHRU de Lille, 2, avenue Oscar-Lambret, 59037 Lille cedex, France.
authors.forename: Nathalie
authors.initials: N
authors.lastname: Grardel
authors.affiliation: Service de réanimation pédiatrique, hôpital Jeanne-de-Flandre, CHU de Lille, 54, avenue Eugène-Avinée, 59037 Lille cedex, France; Université Lille-Nord de France, 59000 Lille, France.
authors.forename: Astrid
authors.initials: A
authors.lastname: Botte
authors.affiliation: Institut de pathologie, CHRU de Lille, 2, avenue Oscar-Lambret, 59037 Lille cedex, France; Université Lille-Nord de France, 59000 Lille, France.
authors.forename: Marie-Christine
authors.initials: MC
authors.lastname: Copin
mesh_terms: D001853:Bone Marrow / Q000473:pathology / Q000821:virology; D018414:CD8-Positive T-Lymphocytes / Q000473:pathology* / Q000821:virology; D002648:Child; D002999:Clone Cells / Q000473:pathology / Q000821:virology; D004279:DNA, Viral / Q000097:blood; D020031:Epstein-Barr Virus Infections / Q000175:diagnosis / Q000473:pathology*; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007121:Immunocompetence; D007244:Infectious Mononucleosis / Q000150:complications; D008198:Lymph Nodes / Q000473:pathology / Q000821:virology; D051359:Lymphohistiocytosis, Hemophagocytic / Q000209:etiology; D008232:Lymphoproliferative Disorders / Q000175:diagnosis / Q000473:pathology* / Q000821:virology; D009102:Multiple Organ Failure / Q000209:etiology; D016133:Polymerase Chain Reaction; D019562:Viral Load; D014766:Viremia / Q000175:diagnosis / Q000473:pathology*
chemical_list: D004279:DNA, Viral
keywords: Child; Enfant; Epstein-Barr virus; Hemophagocytic syndrome; Lymphoprolifération T; Syndrome d’activation macrophagique; T-cell lymphoproliferation