Familial alloimmune neutropenia of NA-2 specificity.
Three siblings with alloimmune neonatal neutropenia are presented. An immunoglobulin G (IgG) antineutrophil antibody specific for NA-2 antigen was demonstrated in maternal serum and in both of the three affected infants who were studied as neonates. The mother's neutrophils were negative for NA-2 antigen. The father and all four children, including the three known to be affected, had neutrophils that expressed NA-2 antigen. One patient studied sequentially demonstrated an inverse relationship between the antineutrophil antibody titer and the absolute neutrophil count. Exchange transfusions did not appear to benefit two of the infants.
Ríos
E
E
Department of Pediatrics, Calvo Mackenna Children's Hospital, Santiago, Chile.
Heresi
G
G
Arevalo
M
M
D000918:Antibody Specificity; D005078:Exchange Transfusion, Whole Blood; D005260:Female; D006801:Humans; D007112:Immunity, Maternally-Acquired; D007114:Immunization; D016867:Immunocompromised Host; D007074:Immunoglobulin G / Q000032:analysis*; D007231:Infant, Newborn; D007518:Isoantibodies / Q000032:analysis*; D007519:Isoantigens / Q000235:genetics / Q000276:immunology*; D008297:Male; D009503:Neutropenia / Q000151:congenital* / Q000235:genetics / Q000276:immunology / Q000628:therapy; D009504:Neutrophils / Q000276:immunology*; D010375:Pedigree; D011247:Pregnancy / Q000276:immunology
D007074:Immunoglobulin G; D007518:Isoantibodies; D007519:Isoantigens; C072753:neutrophil-specific antigen NA2