title: Neutrophil function in chronic neutrophilic leukemia: defective respiratory burst in response to phorbol esters.
abstract: Functional analyses were performed on neutrophils isolated from 6 patients from two institutions who displayed features of chronic neutrophilic leukemia (CNL). These neutrophils demonstrated a consistent deficiency (44 +/- 8% of control values) in superoxide anion (O2-) production in response to the phorbol ester, phorbol myristate acetate (PMA). O2- production in response to chemotactic peptides was near normal (82.3 +/- 10.7% of control values). Bacterial killing was normal in the two patients studied, and chemotaxis was diminished in response to zymosan-activated plasma and to high concentrations of chemotactic peptides in the patients studied. Cytosolic C kinase activity was decreased in one of the two patients studied. These results suggest that a deficient O2- release in response to PMA is a hallmark of neutrophils in CNL and may provide a diagnostic indicator of this condition.
authors.affiliation: Department of Pathology, University of Pittsburgh, Pa.
authors.forename: S S
authors.initials: SS
authors.lastname: Kaplan
authors.forename: R L
authors.initials: RL
authors.lastname: Berkow
authors.forename: R A
authors.initials: RA
authors.lastname: Joyce
authors.forename: R E
authors.initials: RE
authors.lastname: Basford
authors.forename: J C
authors.initials: JC
authors.lastname: Barton
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D006801:Humans; D007700:Kinetics; D015467:Leukemia, Neutrophilic, Chronic / Q000097:blood*; D008297:Male; D008875:Middle Aged; D009240:N-Formylmethionine Leucyl-Phenylalanine / Q000494:pharmacology; D009504:Neutrophils / Q000187:drug effects / Q000502:physiology*; D009895:Opsonin Proteins; D016897:Respiratory Burst / Q000187:drug effects*; D013481:Superoxides / Q000097:blood; D013755:Tetradecanoylphorbol Acetate / Q000494:pharmacology*; D015054:Zymosan / Q000494:pharmacology
chemical_list: D009895:Opsonin Proteins; D013481:Superoxides; D009240:N-Formylmethionine Leucyl-Phenylalanine; D015054:Zymosan; D013755:Tetradecanoylphorbol Acetate