The Bottom Line: Idiopathic CD4 lymphocytopenia (ICL) is a rare non–HIV-related syndrome with unclear natural history and prognosis. In spite of the serious complications ICL has on the patients’ quality of life, data on clinical, etiopathological and therapeutic behavior for ICL are very limited.
Summary: ICL is a hematologic condition of increasing importance due to its diverse clinical and pathological spectrum. Molecular studies have shown the presence of mutations involved in lymphocyte development as potential factors that may contribute to ICL occurrence. ICL patients could present either with common infections or really serious malignant conditions. The role of cytokines, especially interleukin-2, has emerged as one of the main possible mechanisms involved in clinical and pathological behavior of ICL. The main therapeutic approaches are controlling life-threatening infections and underlying disorders along with efforts to cure ICL through rising CD4⁺ cell counts using cytokine interventions and transplantation.
References: Gholamin M., Bazi A., Abbaszadegan MR. “Idiopathic lymphocytopenia.” Current Opinion in Hematology 2015; 22(1): 46-52.
Zonios DI., et al. “Idiopathic CD4+ lymphocytopenia: natural history and prognostic factors.” Blood 2008; 112(2): 287-294.