title: Is there an acute-phase response in steady-state sickle cell disease?
abstract: Metabolic and serum changes during steady-state homozygous sickle cell (SS) disease are consistent with an acute-phase response and raise the possibility that inflammation occurs in SS disease even during the steady state. To test this hypothesis, we measured concentrations of acute phase reactants in patients with SS disease, in patients with sickle cell haemoglobin C (SC) disease, and in normal (AA) control subjects. The concentrations of C-reactive protein and serum amyloid A were increased above 10 mg/L and 5 mg/L, respectively (our definition of an acute-phase response) in 18% (26/143) of subjects with SS disease even when they were symptom free, in 17% (6/35) of subjects with SC disease, and in 1% (1/80) of AA controls (p < 0.001). We suggest that subclinical vaso-occlusion may generate a covert inflammatory response and that the cytokine mediators of this response may contribute to the metabolic abnormalities and growth failure in sickle cell disease.
authors.affiliation: Medical Research Council Laboratories Jamaica, Kingston, West Indies.
authors.forename: A
authors.initials: A
authors.lastname: Singhal
authors.forename: J F
authors.initials: JF
authors.lastname: Doherty
authors.forename: J G
authors.initials: JG
authors.lastname: Raynes
authors.forename: K P
authors.initials: KP
authors.lastname: McAdam
authors.forename: P W
authors.initials: PW
authors.lastname: Thomas
authors.forename: B E
authors.initials: BE
authors.lastname: Serjeant
authors.forename: G R
authors.initials: GR
authors.lastname: Serjeant
mesh_terms: D000209:Acute-Phase Proteins / Q000032:analysis*; D000210:Acute-Phase Reaction / Q000097:blood / Q000209:etiology; D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000097:blood* / Q000150:complications; D001809:Blood Viscosity; D016022:Case-Control Studies; D002648:Child; D003430:Cross-Sectional Studies; D005260:Female; D006450:Hemoglobin SC Disease / Q000097:blood*; D006801:Humans; D008297:Male
chemical_list: D000209:Acute-Phase Proteins