Decreased numbers of chemotactic factor receptors in chronic neutropenia with defective chemotaxis: spontaneous recovery from the neutrophil abnormalities during early childhood.
Childhood chronic neutropenia with decreased numbers of chemotactic factor receptors as well as defective chemotaxis was first demonstrated in an 8-month-old girl. Chemotactic factor receptors on neutrophils were assayed using tritiated N-formyl-methionyl-leucyl-phenylalanine (3H-FMLP). The patient's neutrophils had decreased numbers of the receptors: numbers of the receptors were 20,000 (less than 3 SD) as compared with those of control cells of 52,000 +/- 6,000 (mean +/- SD) (n = 10). The neutropenia disappeared spontaneously by 28 months of age parallel with the improvement of chemotaxis and increase in numbers of chemotactic factor receptors. These results demonstrate a transient decrease of neutrophil chemotactic factor receptors as one of the pathophysiological bases of a transient defect of neutrophil chemotaxis in this disorder.
D000380:Agranulocytosis / Q000378:metabolism*; D002465:Cell Movement; D002908:Chronic Disease; D005260:Female; D006801:Humans; D007223:Infant; D009240:N-Formylmethionine Leucyl-Phenylalanine / Q000378:metabolism; D009503:Neutropenia / Q000378:metabolism*; D009504:Neutrophils / Q000166:cytology; D044042:Receptors, Formyl Peptide; D011971:Receptors, Immunologic / Q000032:analysis*; D014316:Tritium
D044042:Receptors, Formyl Peptide; D011971:Receptors, Immunologic; D014316:Tritium; D009240:N-Formylmethionine Leucyl-Phenylalanine