부산대학교 도서관

A comprehensive evaluation of immune parameters while screening for inborn errors of immunity (IEIs) provides valuable information about the underlying defect. In 2019, the ICMR‐National Institute of Immunohaematology adopted a screening panel modified from the Euroflow consortium‐recommended PIDOT tube, the difference being the addition of HLA‐DR. In the present study, we evaluate the utility of HLA‐DR in our screening panel. The HLA‐DR expression on CD3 + T, its CD4+ and CD8+ subsets, natural killer (NK) and double‐negative T cells (DNT) was compared between patients and controls. HLA‐DR expression on CD3+ T cells or its subsets was significantly elevated in SCID and Omenn's phenotype (P <.001), combined immunodeficiencies (P <.05) including DOCK8 and WAS, diseases of immune dysregulation including FHL (P <.0001) and ALPS (P <.05) and CVID with complications (P <.05). An absence of HLA‐DR marker can help in identifying cases of MHC II deficiency at the time of screening including atypical cases which present without reduction in CD4+ T cell counts and reversal of CD4+/CD8+ ratio. ROC analysis revealed a cut‐off of 14.62%, 19.11% and 1.5% for percentage HLA‐DR expression on CD3+ and CD8+ T cells and HLA‐DR on CD8/HLA‐DR on CD4, respectively, for FHL. Random forest analysis identified HLA‐DR (%) on NK cells, ratio of HLA‐DR CD8+/HLA‐DR CD4+, NK cell (%), T cell (%), HLA‐DR+ CD8(%) and CD45pos NK cell (%) as important predictors for FHL. Evaluation of HLA‐DR expression during screening can provide corroborative clues to the diagnosis of underlying IEI along with other immunological abnormalities. [ABSTRACT FROM AUTHOR]