title: Abnormal T cell activation and skewed T cell receptor V beta repertoire usage in Japanese patients with idiopathic portal hypertension.
abstract: Idiopathic portal hypertension (IPH), a disorder of unknown etiology, is characterized by a noncirrhotic portal hypertension associated with splenomegaly, hypersplenism, and anemia. We examined the surface phenotypes of T cells and the T cell receptor V beta repertoire in patients with IPH. The T cells in peripheral blood samples and from spleens showed a marked increase in frequencies of HLA-DP(+)- and HLA-DR(+)-activated T cells and the observed high frequencies in the blood were to a considerable extent reduced after splenectomy. Thus, the continuous activation of T cells may occur initially in the spleen. Investigation of T cell receptor V beta repertoire revealed a significant skewing of V beta 9 and V beta 11 in both peripheral blood and splenic T cells and V beta 12 in splenic T cells. The IPH may be a disease mediated by a continuous stimulation with either a certain antigen or more likely a superantigen.
authors.affiliation: Department of Pathology, Juntendo University School of Medicine, Tokyo, Japan.
authors.forename: K
authors.initials: K
authors.lastname: Tokushige
authors.forename: S
authors.initials: S
authors.lastname: Hirose
authors.forename: H
authors.initials: H
authors.lastname: Nishimura
authors.forename: M
authors.initials: M
authors.lastname: Fujimori
authors.forename: K
authors.initials: K
authors.lastname: Yamauchi
authors.forename: H
authors.initials: H
authors.lastname: Obata
authors.forename: S
authors.initials: S
authors.lastname: Futagawa
authors.forename: T
authors.initials: T
authors.lastname: Shirai
mesh_terms: D000328:Adult; D005260:Female; D015333:Gene Rearrangement, beta-Chain T-Cell Antigen Receptor; D006801:Humans; D006975:Hypertension, Portal / Q000276:immunology*; D008213:Lymphocyte Activation; D008297:Male; D008875:Middle Aged; D016693:Receptors, Antigen, T-Cell, alpha-beta / Q000235:genetics*; D013156:Splenectomy; D016176:T-Lymphocyte Subsets / Q000276:immunology; D013601:T-Lymphocytes / Q000276:immunology*
chemical_list: D016693:Receptors, Antigen, T-Cell, alpha-beta