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Markers of endothelial dysfunction and leucocyte activation in Saudi and non-Saudi haplotypes of sickle cell disease.
Ann Hematol 2017; 96(1):141-146AH

Abstract

Sickle cell disease (SCD) is an autosomal recessive inherited hemoglobinopathy, characterized by chronic hemolysis and recurrent vaso-occlusive crisis (VOC). This study investigates changes in leucocyte subsets and the relationship between cell adhesion molecule expression and disease manifestations in patients during steady state and acute VOC. We compared soluble E-selectin and P-selectin levels in 84 SCD patients, in steady state and during VOC to 84 healthy controls. Using immunophenotyping, we also compared lymphocyte subsets in these three groups. Further, we compared E-selectin and P-selectin levels in patients of Saudi ethnicity to non-Saudi patients, in all three groups. Lymphocyte subsets showed high percentages of total T lymphocytes, T helper and suppressor lymphocytes, B lymphocytes as well as NK cells in patients with SCD during steady state, while B lymphocytes and NK cells were significantly higher during acute VOC crisis. High levels of both soluble E-selectin (sE-selectin) and soluble P-selectin (sP-selectin) markers were demonstrated in the serum of patients with SCD during both steady state and acute VOC. Levels of selectins were significantly higher in acute VOC. The immunophenotypic expression of L-selectin, on leucocytes, was high in SCD both during steady state and during acute VOC in comparison to normal control subjects. There was no significant difference in all three study groups between Saudi and non-Saudi patients. These findings suggest that patients with SCD have increased expression of adhesion molecules: E-selectin and P-selectin, which play an important role in the pathogenesis of VOC. Despite the distinct phenotype of Saudi patients with SCD, there was no significant difference in levels of soluble E-selectin and soluble P-selectin between Saudi and non-Saudi patients in all three groups. While sickle cell disease is a well-recognized state of chronic inflammation, the role of specific adhesion molecules is steadily unraveling. Studies are underway to investigate the potential role of selectin antagonists, for prevention and reversal of acute vascular occlusions in SCD patients.

Authors+Show Affiliations

Department of Hematology, King Abdulaziz University, Jeddah, SA, Saudi Arabia.Department of Hematology, King Abdulaziz University, Jeddah, SA, Saudi Arabia. sadam@kau.edu.sa. Duke University Medical Center, Durham, NC, USA. sadam@kau.edu.sa.Manchester Metropolitan University, Manchester, UK.Department of Hematology, King Abdulaziz University, Jeddah, SA, Saudi Arabia.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

27686084

Citation

Al Najjar, Salwa, et al. "Markers of Endothelial Dysfunction and Leucocyte Activation in Saudi and non-Saudi Haplotypes of Sickle Cell Disease." Annals of Hematology, vol. 96, no. 1, 2017, pp. 141-146.
Al Najjar S, Adam S, Ahmed N, et al. Markers of endothelial dysfunction and leucocyte activation in Saudi and non-Saudi haplotypes of sickle cell disease. Ann Hematol. 2017;96(1):141-146.
Al Najjar, S., Adam, S., Ahmed, N., & Qari, M. (2017). Markers of endothelial dysfunction and leucocyte activation in Saudi and non-Saudi haplotypes of sickle cell disease. Annals of Hematology, 96(1), pp. 141-146. doi:10.1007/s00277-016-2823-7.
Al Najjar S, et al. Markers of Endothelial Dysfunction and Leucocyte Activation in Saudi and non-Saudi Haplotypes of Sickle Cell Disease. Ann Hematol. 2017;96(1):141-146. PubMed PMID: 27686084.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Markers of endothelial dysfunction and leucocyte activation in Saudi and non-Saudi haplotypes of sickle cell disease. AU - Al Najjar,Salwa, AU - Adam,Soheir, AU - Ahmed,Nessar, AU - Qari,Mohamed, Y1 - 2016/09/30/ PY - 2016/08/13/received PY - 2016/09/08/accepted PY - 2016/10/1/pubmed PY - 2017/2/2/medline PY - 2016/10/1/entrez KW - Endothelial activation KW - Sickle cell disease KW - Soluble selectin SP - 141 EP - 146 JF - Annals of hematology JO - Ann. Hematol. VL - 96 IS - 1 N2 - Sickle cell disease (SCD) is an autosomal recessive inherited hemoglobinopathy, characterized by chronic hemolysis and recurrent vaso-occlusive crisis (VOC). This study investigates changes in leucocyte subsets and the relationship between cell adhesion molecule expression and disease manifestations in patients during steady state and acute VOC. We compared soluble E-selectin and P-selectin levels in 84 SCD patients, in steady state and during VOC to 84 healthy controls. Using immunophenotyping, we also compared lymphocyte subsets in these three groups. Further, we compared E-selectin and P-selectin levels in patients of Saudi ethnicity to non-Saudi patients, in all three groups. Lymphocyte subsets showed high percentages of total T lymphocytes, T helper and suppressor lymphocytes, B lymphocytes as well as NK cells in patients with SCD during steady state, while B lymphocytes and NK cells were significantly higher during acute VOC crisis. High levels of both soluble E-selectin (sE-selectin) and soluble P-selectin (sP-selectin) markers were demonstrated in the serum of patients with SCD during both steady state and acute VOC. Levels of selectins were significantly higher in acute VOC. The immunophenotypic expression of L-selectin, on leucocytes, was high in SCD both during steady state and during acute VOC in comparison to normal control subjects. There was no significant difference in all three study groups between Saudi and non-Saudi patients. These findings suggest that patients with SCD have increased expression of adhesion molecules: E-selectin and P-selectin, which play an important role in the pathogenesis of VOC. Despite the distinct phenotype of Saudi patients with SCD, there was no significant difference in levels of soluble E-selectin and soluble P-selectin between Saudi and non-Saudi patients in all three groups. While sickle cell disease is a well-recognized state of chronic inflammation, the role of specific adhesion molecules is steadily unraveling. Studies are underway to investigate the potential role of selectin antagonists, for prevention and reversal of acute vascular occlusions in SCD patients. SN - 1432-0584 UR - https://www.unboundmedicine.com/medline/citation/27686084/Markers_of_endothelial_dysfunction_and_leucocyte_activation_in_Saudi_and_non_Saudi_haplotypes_of_sickle_cell_disease_ L2 - https://dx.doi.org/10.1007/s00277-016-2823-7 DB - PRIME DP - Unbound Medicine ER -