title: Increased circulating levels of soluble HLA class I heterodimers in patients with sickle cell disease.
abstract: This study examined the presence of a persistent state of low-grade inflammation in sickle cell anemia patients by measuring circulating sHLA-I heterodimers and C-reactive protein during the steady state and after recent crises. Thirty-nine pediatric sickle hemoglobinopathy patients were studied during the steady state and 11 patients were evaluated within 1 month of a painful crisis. A disease severity score was generated for each patient, and soluble HLA-I (sHLA-I) and C-reactive protein levels were determined. Soluble HLA-I was significantly elevated in 55% of the steady-state group and in 36% of the recent-crisis group. The percentage of patients with elevated sHLA-I differed in the various disease subgroups in the steady state: 46% of Hb SS patients, 70% of Hb SC patients, 75% of Hb S beta-thal patients, and 20% of Hb SSF patients. Steady-state and recent-crisis sHLA-I levels were not significantly different. C-reactive protein levels were elevated in 11% of steady-state patients and in 9% of recent-crisis patients. Soluble HLA-I levels did not correlate with C-reactive protein levels or disease severity score, age, hemoglobin, reticulocyte count, platelet count, or white cell count. These results show that the majority of sickle hemoglobinopathy patients have elevated sHLA-I levels during the steady state and after recent crisis, suggesting the presence of chronic inflammation during the steady state.
authors.affiliation: Department of Pediatrics, Louisiana State University Medical Center, New Orleans 70112-2822, USA.
authors.forename: C
authors.initials: C
authors.lastname: Moore
authors.forename: M
authors.initials: M
authors.lastname: Ehlayel
authors.forename: J
authors.initials: J
authors.lastname: Inostroza
authors.forename: L E
authors.initials: LE
authors.lastname: Leiva
authors.forename: S
authors.initials: S
authors.lastname: Kuvibidila
authors.forename: L
authors.initials: L
authors.lastname: Yu
authors.forename: R
authors.initials: R
authors.lastname: Gardner
authors.forename: D L
authors.initials: DL
authors.lastname: Ode
authors.forename: R
authors.initials: R
authors.lastname: Warrier
authors.forename: R U
authors.initials: RU
authors.lastname: Sorensen
mesh_terms: D000755:Anemia, Sickle Cell / Q000097:blood* / Q000276:immunology; D002097:C-Reactive Protein / Q000032:analysis; D002648:Child; D005260:Female; D015395:Histocompatibility Antigens Class I / Q000097:blood*; D006801:Humans; D008297:Male; D012720:Severity of Illness Index
chemical_list: D015395:Histocompatibility Antigens Class I; D002097:C-Reactive Protein