Abstract
Idiopathic CD4 lymphocytopenia (ICL) is a rare entity of immune deficit of CD4+ T cells (below 300/mm3) which was first defined in 1992, unrelated to human immunodeficiency virus (HIV) syndrome without predefined clinical presentation and natural history. The etiology, pathogenesis, and management of ICL remains poorly understood and inadequately defined. The clinical presentation can range from serious opportunistic infections to incidentally diagnosed asymptomatic individuals. Cryptococcal and nontuberculous mycobacterial infections and progressive multifocal leukoencephalopathy are the most significant presenting infections, although the spectrum of opportunistic diseases can be similar to that in patients with lymphopenia and HIV infection. The prognosis is influenced by the accompanying opportunistic infections and response to the treatment. This rare disease invites uncountable opportunistic infections sometimes leading to lethal outcome. We report a case of ICL in an immunocompetent 18-year-old male with a history of intermittent epistaxis, fungal nasal polyp diagnosed to have Rhizopus with drug-induced acute kidney injury during the course of treatment, a rarest diagnosis as underlying main disease entity. To the best of our knowledge, ICL presenting with opportunistic infection of Rhizopus is the first case to be reported here from a tertiary care center of India, a developing country where a major population lives with poor hygiene and low socioeconomic status.
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