Successful treatment of chronic autoimmune neutropenia with cyclosporin A.
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.
Martino
R
R
Departament d'Hematologia, Unitat d'Hematologia Clínica, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain.
Sureda
A
A
Ayats
R
R
Muñiz-Díaz
E
E
Altés
A
A
Brunet
S
S
D000328:Adult; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology; D001327:Autoimmune Diseases / Q000150:complications / Q000188:drug therapy*; D002908:Chronic Disease; D016572:Cyclosporine / Q000008:administration & dosage / Q000627:therapeutic use*; D005334:Fever / Q000150:complications / Q000188:drug therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage / Q000627:therapeutic use*; D008297:Male; D009503:Neutropenia / Q000150:complications / Q000188:drug therapy*; D009504:Neutrophils / Q000276:immunology; D019226:Oral Ulcer / Q000150:complications / Q000188:drug therapy; D011241:Prednisone / Q000008:administration & dosage / Q000627:therapeutic use
D019268:Antibodies, Antineutrophil Cytoplasmic; D007166:Immunosuppressive Agents; D016572:Cyclosporine; D011241:Prednisone