ABSTRACT
We report the first infant case with hepatosplenic gammadelta T-cell lymphoma after recurrent acute disseminated encephalomyelitis-like, which were rapidly resolved with steroid pulse therapy. The patient had a history of recurrent bronchitis, intractable diarrhea, and failure to thrive since 4 months of age. Immunologic analysis revealed higher percentage of circulating gammadelta T-cells with markedly reduced numbers of CD3TCRalphabetaCD8 T-cells. The patient developed gammadelta T-cell lymphoma at the age of 15 months. Clinical course of the patient suggests the importance of immunological background for the development of hepatosplenic gammadelta T-cell lymphoma.
Subject(s)
Encephalomyelitis, Acute Disseminated/complications , Liver Neoplasms/complications , Lymphoma, T-Cell/complications , Receptors, Antigen, T-Cell, gamma-delta/analysis , Encephalomyelitis, Acute Disseminated/pathology , Humans , Infant , Lymphoma, T-Cell/immunology , Magnetic Resonance Imaging , Male , Splenic NeoplasmsABSTRACT
OBJECTIVE: To study expression and function of the chemokine receptor CCR5 in synovial fluid (SF) T cells from patients with rheumatoid arthritis (RA). METHODS: Expression of CCR5 was studied by flow cytometry and immunoblotting. The chemotactic response of T cells to chemokines was studied in cell migration assay. Tyrosine phosphorylation of Crk-associated substrate lymphocyte-type (CasL) was evaluated in immunoprecipitation and immunoblotting. RESULTS: SF T cells showed an increase in the population of CCR5, CXCR4, and CD45RO positive cells and exhibited an increase in chemotactic activity, which was not augmented with RANTES but stromal cell-derived factor-1alpha. Tyrosine phosphorylation per CasL molecule was markedly enhanced in SF T cells. In H9 cells, tyrosine phosphorylation of not only focal adhesion kinase but also CasL was induced after treatment with RANTES. Downmodulation of CCR5 by RANTES was decreased and recycling of CCR5 was accelerated in SF T cells when compared with peripheral blood (PB) T cells. When CD45RO positive PB T cells were cultured with interleukin 2, blunted responsiveness to RANTES-induced chemotaxis was reproduced as well as spontaneous chemotaxis, increased expression of CCR5, and aberrant receptor dynamics, after RANTES stimulation as observed in SF T cells. CONCLUSION: Synovial fluid T cells highly positive for CCR5 show aberrant characteristics; resistant to RANTES in terms of migration, but responsive in terms of dynamics of CCR5.
