Increased circulating levels of soluble HLA class I heterodimers in patients with sickle cell disease

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 dur...

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Bibliographic Details
Published in:Journal of the National Medical Association 1998-03, Vol.90 (3), p.157-163
Main Authors: MOORE, C, EHLAYEL, M, INOSTROZA, J, LEIVA, L. E, KUVIBIDILA, S, LOLIE YU, GARDNER, R, ODE, D. L, WARRIER, R, SORENSEN, R. U
Format: Article
Language:English
Subjects:
Anemia, Sickle Cell - blood
Anemia, Sickle Cell - immunology
Anemias. Hemoglobinopathies
Biological and medical sciences
C-Reactive Protein - analysis
Child
Diseases of red blood cells
Female
Hematologic and hematopoietic diseases
Histocompatibility Antigens Class I - blood
Humans
Male
Medical sciences
Severity of Illness Index
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Summary: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.
ISSN:0027-9684
1943-4693