RESUMO
This study investigates HLA-DR expression on activated T cells and serum neopterin levels in Juvenile Dermatomyositis (JDM) children pre- and post-treatment. Sixty-nine JDM children (less than 18 years) were included. Elevated HLA-DR+ T cells (>7 %) were observed in 19 % of untreated cases. Post-treatment, mean HLA-DR+ T cells decreased from 5.1 to 2.9 (P < 0.001), and serum neopterin levels declined from 19.3 to 9.1 nmol/L (P < 0.0001). A positive correlation between serum neopterin and HLA-DR T cell percentage was observed (r = 0.39, P = 0.01). Intravenous steroid treatment exhibited a 47.4 % improvement in HLA-DR+ T cells and a 50.5 % reduction in serum neopterin levels, in contrast to 14.8 % and 34.1 % in the oral steroid group. In conclusion, treatment, particularly with IV steroids, significantly improved HLA-DR+ T cells percentage and neopterin levels. A correlation between HLA-DR+ T cells percentage and serum neopterin was noted in untreated JDM patients.
Assuntos
Transplante de Células , Subunidade gama Comum de Receptores de Interleucina/genética , Mutação , Transdução de Sinais , Linfócitos T/metabolismo , Linfócitos T/transplante , Adulto , Criança , Feminino , Humanos , Imunofenotipagem , Lactente , Masculino , Fenótipo , Linfócitos T/imunologiaRESUMO
The primary immunodeficiency diseases (PIDs) encompass an extremely large and diverse number of clinical disorders caused by mutations in genes that affect virtually every measurable component of our immune systems. Many of the genetic mutations lead to abnormalities that can be detected in circulating peripheral blood cells of suspected patients by flow cytometry and the appropriate combinations of reagents and in vitro manipulations. The flow cytometry procedures that have been developed to detect abnormalities in peripheral blood cells of primary immunodeficiency patients can barely be covered in an entire book, let alone one chapter. Instead of attempting to cover each disease with a specific assay or test, we review three procedures each covering a global aspect of the observed immune abnormality, i.e., detection of lymphocyte subset abnormalities, lymphocyte "marker" abnormalities, and leukocyte function abnormalities.