Abstract
Idiopathic CD4 lymphopenia (ICL) is a heterogeneous syndrome characterized by low CD4 T lymphocyte counts at <300cells/μL on at least two occasions in the absence of a known infection or intercurrent illness. The clinical manifestations of ICL vary greatly, from asymptomatic lymphopenia to severe opportunistic disease, autoimmunity, or neoplasia (frequently related to persistent viral infections). Most common infections reported in ICL include cryptococcal disease, human papilloma, and herpes zoster viruses. The diagnosis of ICL requires exclusion of genetic immune deficiencies and other hematologic diseases or infections, and confirmation of low CD4 lymphocyte counts. Although cytokine therapies and allogeneic stem cell transplantation have been rarely used with variable success, there is currently no specific therapy for ICL. Treatment of underlying infections and antibiotic prophylaxis are the mainstay of available therapy of ICL. A better understanding of the etiology, pathogenesis, and prognosis of ICL will assist in improved therapeutic approaches.
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