Idiopathic CD4+ lymphocytopenia and systemic vasculitis.

Bordin, G; Ballaré, M; Paglino, S; Ravanini, P; Dulio, D; Malosso, M C; Boldorini, R; Monteverde, A · J Intern Med · 1996

case_report · Level V

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Abstract

The syndrome defined as "idiopathic CD4 lymphocytopenia' (ICL) is a rare disease of unknown aetiology, often associated with severe depression of immune defences and the occurrence of opportunistic infections. A case is reported wherein a severe immunodeficiency syndrome with persistent idiopathic CD4+ lymphopenia developed in a woman suffering from systemic microscopic polyarteritis; no signs of HIV 1/2 or HTLV I/II infection were evident. The patient died of widespread opportunistic infections. The association of ICL with vasculitis has never been reported until now. A link between the two diseases cannot be ruled out.

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