Document Detail

Clonal dissemination of T-lymphocytes in scid mice from familial hemophagocytic lymphohistiocytosis.
MedLine Citation:
PMID:  10064188     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: Although familial hemophagocytic lymphohistiocytosis (FHL) has been considered a disorder of T-cell dysfunction, there is no evidence of the clonal origin of T-cells in this disease. PROCEDURE: We engrafted mononuclear cells (MNCs) from five FHL patients into scid mice and examined the infiltration of human cells in mouse organs. The characterization of human cells that infiltrated in the mouse organs was then performed. RESULTS: A diffuse infiltration of human lymphoid cells was detected in scid mice treated with 1 x 10(6) MNCs from one of the five patients. These cells were positive for HLA-DR and CD3, but negative for CD4, CD8, CD20, and CD68, suggesting the infiltration of double negative (DN) T-cells. The MNCs from the other four patients induced murine lymphoma-like disease; T-cell lymphoma in one and lymphoma of unknown origin in three. The characterization of these human DN T-cells was performed. The analysis of the Vbeta repertoire showed no preferential usage of the Vbeta family in MNCs, while the dominant expression of Vbeta13 was detected in T-cells infiltrating in the spleen and lung. A Jbeta analysis showed the restricted usage of Jbeta1.2 for Vbeta13 in these cells, and the clonality of Vbeta13-Jbeta1.2 fragment was confirmed by a single-strand confirmation polymorphism analysis. The analysis of the Valpha repertoire showed that Valpha24 was exclusively used in these DN T-cells, but no usage of JalphaQ for Valpha24 was observed. CONCLUSIONS: A clonal expansion of T-cells was induced in scid mice by the engraftment of MNCs from an FHL patient. The infiltration of DN alphabeta T-cells bearing invariant Valpha24 T-cell receptor in mouse organs may provide a useful clue to the pathogenesis of FHL. In the patients whose MNCs induced murine lymphoma-like disease, some cytokines or unknown factors that stimulate the growth and the tumorigenicity of murine lymphocytes might be produced by the MNCs engrafted in scid mice. Further study is needed to confirm the validity of our experimental approach and the findings observed in scid mice by using more FHL samples.
E Ishii; N Yoshida; N Kimura; J Fujimoto; S Mizutani; M Sako; S Hibi; M Nagano; T Yoshida; T Mori; N Kiyokawa; S Mohri; T Tanaka; S Miyazaki; T Hara
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Medical and pediatric oncology     Volume:  32     ISSN:  0098-1532     ISO Abbreviation:  Med. Pediatr. Oncol.     Publication Date:  1999 Mar 
Date Detail:
Created Date:  1999-03-18     Completed Date:  1999-03-18     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  7506654     Medline TA:  Med Pediatr Oncol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  201-8     Citation Subset:  IM    
Division of Pediatrics, Hamanomachi Hospital, Fukuoka, Japan.
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MeSH Terms
Clone Cells
Histiocytosis, Non-Langerhans-Cell / genetics,  pathology*
Immunoglobulin Variable Region / genetics
Leukocytes, Mononuclear / immunology,  transplantation
Lymphocyte Activation / genetics
Mice, SCID
Receptors, Antigen, T-Cell, alpha-beta / metabolism
T-Lymphocytes / immunology,  pathology*,  physiology
Transplantation, Heterologous
Reg. No./Substance:
0/Immunoglobulin Variable Region; 0/Receptors, Antigen, T-Cell, alpha-beta

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