title: Congenital autoimmune neutropenia in two premature neonates.
abstract: Autoimmune neutropenia (AIN) has been reported in infants and children, but not in neonates. AIN is caused by antibodies produced by the patient against their own neutrophils; therefore, it differs from the more common alloimmune neonatal neutropenia and the neonatal neutropenia because of a maternal autoimmune disease in which antineutrophil antibodies of maternal origin cross the placenta. We observed 2 cases of congenital AIN in premature neonates. These are the youngest reported cases, and indicate that AIN can have a prenatal onset. Examination of the bone marrow biopsies revealed an increase in B lymphocytes and myeloperoxidase-positive cells with a maturation arrest at the myelocyte stage. Recombinant human granulocyte colony-stimulating factor effectively treated the neutropenia, as it does in infantile AIN. Ten months after the diagnosis, 1 of the patients still requires recombinant human granulocyte colony-stimulating administration.
authors: Gainesville, FL 32610-0296, USA. calhoda@peds.ufl.edu
authors: D A
authors: DA
authors: Calhoun
authors: L M
authors: LM
authors: Rimsza
authors: D J
authors: DJ
authors: Burchfield
authors: M
authors: M
authors: Millsaps
authors: R D
authors: RD
authors: Christensen
authors: J
authors: J
authors: Budania
authors: J
authors: J
authors: McCullough
mesh_terms: D001327:Autoimmune Diseases / Q000151:congenital* / Q000188:drug therapy / Q000473:pathology; D001706:Biopsy; D001853:Bone Marrow / Q000473:pathology; D005260:Female; D016179:Granulocyte Colony-Stimulating Factor / Q000627:therapeutic use; D006801:Humans; D007231:Infant, Newborn; D007235:Infant, Premature, Diseases / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology; D008297:Male; D009503:Neutropenia / Q000151:congenital* / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology; D011994:Recombinant Proteins
chemical_list: D011994:Recombinant Proteins; D016179:Granulocyte Colony-Stimulating Factor