Downregulation of CD5 and dysregulated CD8 T-cell activation.

CD5 is a cell surface molecule that is expressed on most circulating T cells and a small population of B cells, and is involved in modulation of antigen-specific receptor-mediated activation. Downregulation of CD5 on CD8 T cells is a poorly understood but increasingly recognized phenomenon that may be associated with dysregulated T-cell activation. An increased subpopulation of activated CD8 T cells with downregulation of CD5 has recently been described in patients with Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (HLH) and familial HLH caused by perforin deficiency and Munc 13-4 deficiency. These cells were detectable only in the acute phase of HLH, in which patients exhibited hypercytokinemia, and declined progressively after successful treatment in parallel with improvement of systemic inflammation. It is unknown whether CD8 T cells from HLH with other causes have similar profiles. Assessment of CD5 expression on T cells has the potential to assist in the understanding of the diagnosis and pathogenesis of human inflammatory diseases such as HLH.