Chronic primary autoimmune neutropenia (AIN) is a distinct clinical en
tity seen mostly in young children, characterized by persistent neutro
penia with circulating anti-neutrophil antibodies and no associated di
sorders known to produce AIN. Herein we report a 22-year-old male who
spontaneously developed severe chronic neutropenia with recurrent epis
odes of high fever and oral aphthous ulcers. Laboratory evaluations de
tected the presence of anti-granulocyte autoantibodies directed agains
t the NA1 neutrophil-specific antigen. Clinical, laboratory and roentg
enographic 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 prednison
e and high-dose cyclosporin A.