NIH researchers uncover new details on rare immune disease. NIH researchers characterize ICL, a rare immune deficiency, linking severe cases to higher risk of infections and cancers. In an 11-year study, researchers at the National Institutes of Health (NIH) have further characterized idiopathic
Scanning electron micrograph of a human T cell from the immune system of a healthy donor. In an 11-year study by the National Institutes of Health, researchers further characterized idiopathic CD4 lymphocytopenia , a rare immune deficiency that increases vulnerability to infectious diseases, autoimmune diseases, and cancers. Credit: NIAIDNIH researchers characterize ICL, a rare immune deficiency, linking severe cases to higher risk of infections and cancers.
ICL is a condition marked by too few CD4+ T-cells, which are a type of white blood cell. The clinical definition of ICL is a CD4+ T-cell count of less than 300 cells per cubic millimeter of blood for at least six weeks, in the absence of any disease or therapy associated with reduced white blood cells. Unlike HIV, athat suppresses the immune system if left untreated, there is no evidence that ICL is transmitted from person to person, and it has no known cause.
In this observational study, the NIAID researchers quantified immune cells and noted the presence of opportunistic infections—infections that typically only affect people with suppressed immune systems—and other clinical conditions among 91 participant volunteers with ICL. The most prevalent opportunistic infections were human papillomavirus-related diseases , cryptococcosis , molluscum contagiosum , and mycobacterial diseases other than tuberculosis .
These findings further support the inverse correlation between CD4+ T-cell count and susceptibility to viral, fungal, and mycobacterial infections, as well as certain cancers, according to the authors. NIAID continues to pursue research on the natural history of rare conditions such as ICL to understand disease progression, as well as potential therapeutic interventions.
“Reappraisal of Idiopathic CD4 Lymphocytopenia at 30 Years” by Andrea Lisco, M.D., Ph.D., Ana M. Ortega-Villa, Ph.D., Harry Mystakelis, M.D., M.H.S., Megan V. Anderson, R.N., B.A., Allyson Mateja, M.S.P.H., Elizabeth Laidlaw, P.A.-C., Maura Manion, M.D., Gregg Roby, R.N., B.S., Jeanette Higgins, Ph.D., Safia Kuriakose, Pharm.D., Magdalena A. Walkiewicz, Ph.D., Morgan Similuk, Sc.M., Jennifer W. Leiding, M.D., Alexandra F. Freeman, M.D., Virginia Sheikh, M.D. and Irini Sereti, M.D., M.H.S.
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