title: Oligoclonal expansion of alphabeta T lymphocytes in Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis with abnormal karyotypes.
abstract: We observed a fatal case of Epstein-Barr virus (EBV)-associated hemophagocytic lymphohistiocytosis (HLH) with an abnormal karyotype. Increased levels of alphabeta T cells of the patient were investigated using an inverse polymerase chain reaction (PCR) of the T-cell receptor variable region gene, followed by Jbeta-PCR and single-strand conformation polymorphism (SSCP) to confirm the clonality of specific alphabeta-T cell subsets. A high frequency (>15%) was recognized in Vbeta9 at onset, but not in any Vbeta and Valpha families 2 weeks after chemotherapy. High levels (>20%) of some Jbeta genes were detected in all Vbeta families investigated, and the predominant bias of the Jbeta2 gene relative to the Jbeta1 gene (86.1% versus 13.9% at onset, and 77.4% versus 23.5% after chemotherapy) was recognized in pan-alphabeta T cells. When each Vbeta-Jbeta fragment was compared among the samples at onset and after chemotherapy by SSCP analysis, several distinct bands were observed that indicate a clonal evolution. Thus, the findings suggest that some of the alphabeta T cell clones could be associated with abnormal karyotypes in EBV-HLH. The present findings provide molecular evidence of the presence of oligoclonal T cells in pan-alphabeta-T cells and clonal evolution during a short clinical course in EBV-HLH with abnormal karyotypes.
authors.affiliation: Division of Pediatrics, Hamanomachi Hospital, Fukuoka, Japan. ishiei@post.saga.med.ac.jp
authors.forename: E
authors.initials: E
authors.lastname: Ishii
authors.forename: N
authors.initials: N
authors.lastname: Kimura
authors.forename: K
authors.initials: K
authors.lastname: Honda
authors.forename: M
authors.initials: M
authors.lastname: Eguchi
authors.forename: H
authors.initials: H
authors.lastname: Nakayama
authors.forename: M
authors.initials: M
authors.lastname: Tanaka
authors.forename: I
authors.initials: I
authors.lastname: Ichinose
authors.forename: T
authors.initials: T
authors.lastname: Yoshida
authors.forename: K
authors.initials: K
authors.lastname: Tamura
mesh_terms: D002648:Child; D002869:Chromosome Aberrations; D004854:Herpesvirus 4, Human; D015616:Histiocytosis, Non-Langerhans-Cell / Q000235:genetics* / Q000276:immunology; D006801:Humans; D007621:Karyotyping; D008297:Male; D016133:Polymerase Chain Reaction; D016693:Receptors, Antigen, T-Cell, alpha-beta / Q000032:analysis*; D013601:T-Lymphocytes / Q000502:physiology*
chemical_list: D016693:Receptors, Antigen, T-Cell, alpha-beta