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Small Populations of Normal Cells Affect Immunity in Patients with XLP1

News   Nov 12, 2018 | Original Story from the Tokyo Medical and Dental University.

 
Small Populations of Normal Cells Affect Immunity in Patients with XLP1

This slide illustrates a model of cellular and humoral immunity regarding SAP. In normal individuals, SAP+ CD8+ T cells attack EBV-infected B cells via IFN-gamma, perforin, and granzyme, and EBV-infected B cells lead to cell death. SAP+ CD4+ T cells help immunoglobulin production from B cells via IL-10 and ICOS. In XLP1 patients, the SAP function is completely impaired, and the patients develop FIM or HLH and hypo- or dysgammglobulinemia. In XLP1 patients with somatic reversion, as demonstrated in this study, small populations of SAP+ T cells can rescue the function. The patients were not associated with FIM or HLH, and they presented with IgG3 and IgG4 deficiency only. Credit: Department of Child Health and Development,TMDU.

 
 
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