Successful treatment of chronic autoimmune neutropenia with cyclosporin A

Abstract

Chronic primary autoimmune neutropenia (AIN) is a distinct clinical entity seen mostly in young children, characterized by persistent neutropenia with circulating anti-neutrophil antibodies and no associated disorders known to produce AIN. Herein we report a 22-year-old male who spontaneously developed severe chronic neutropenia with recurrent episodes of high fever and oral aphthous ulcers. Laboratory evaluations detected the presence of anti-granulocyte autoantibodies directed against the NA1 neutrophil-specific antigen. Clinical, laboratory and roentgenographic testing did not reveal any disorder known to be associated with AIN. The patient' s severe neutropenia did not respond to therapy with prednisone alone, but resolved following treatment with prednisone and high-dose cyclosporin A.