title: Epstein-Barr virus induced virus-associated hemophagocytic syndrome and monoclonal TCR-beta rearrangement: a case report.
abstract: Virus-associated hemophagocytic syndrome (VAHS) is a rare histiocytic proliferative disorder secondary to viral infection affecting children and young adults and in which there is both a poor prognosis and no specific treatment. We report a case of VAHS in a 22-year-old woman secondary to infection with Epstein Barr virus in which multiorgan failure developed with a fatal outcome 3 weeks after presentation. Clonal analysis showed EBV incorporation into the T cell genome accompanied by monoclonal rearrangement of the TCR beta gene. Treatment with cyclosporin A resulted in hematological improvement but the patient died of disseminated fungal infection. We propose that bone marrow ablation and allografting should be available for these rare cases and suggest that a protocol should be developed with designated transplant centres to aid early referral.
authors.affiliation: Department of Medicine, Maidstone Hospital, Kent, U.K.
authors.forename: G
authors.initials: G
authors.lastname: Bird
authors.forename: D
authors.initials: D
authors.lastname: Peel
authors.forename: K
authors.initials: K
authors.lastname: McCarthy
authors.forename: H
authors.initials: H
authors.lastname: Williams
mesh_terms: D000328:Adult; D016572:Cyclosporine / Q000627:therapeutic use; D005260:Female; D015333:Gene Rearrangement, beta-Chain T-Cell Antigen Receptor; D006566:Herpesviridae Infections / Q000150:complications*; D004854:Herpesvirus 4, Human; D015616:Histiocytosis, Non-Langerhans-Cell / Q000188:drug therapy / Q000235:genetics* / Q000821:virology*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008213:Lymphocyte Activation; D016133:Polymerase Chain Reaction; D013601:T-Lymphocytes / Q000502:physiology / Q000648:ultrastructure; D014412:Tumor Virus Infections / Q000150:complications*
chemical_list: D007166:Immunosuppressive Agents; D016572:Cyclosporine