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The intriguing contribution of white blood cells to sickle cell disease - a red cell disorder.
Blood Rev 2004; 18(1):65-73BR

Abstract

Sickle cell disease (SCD) is characterized by a point mutation that replaces adenine with thymidine in the sixth codon of the beta-globin gene, a unique morphological abnormality of red blood cells, vaso-occlusion with ischaemic tissue injury, and susceptibility to infections. Vascular lumen obstruction in SCD results from interaction of erythrocytes, leukocytes, platelets, plasma proteins, and the vessel wall. The disease phenotype is a product of various genes and environmental factors acting in concert with the protein lesion underlying the red cell anomaly. The severity of SCD increases with leukocyte count. The biological basis and therapeutic implications of this relationship are discussed. Leukocytes contribute to SCD by adhering to blood vessel walls and obstructing the lumen, aggregating with other blood cells with more effective blockage of the lumen, stimulating the vascular endothelium to increase its expression of ligands for adhesion molecules on blood cells, and causing tissue damage and inflammatory reaction which predispose to vaso-occlusion. Patients with impaired ability of leukocytes to kill microbes are more prone to infections; which precipitate sickle cell crisis. Reduction of leukocyte count ameliorates SCD. Similarly, targeted blockade or reduced synthesis of specific leukocyte adhesion molecules and their ligands might confer clinical benefit in SCD.

Authors+Show Affiliations

Haematology Department, St. Thomas Hospital, Lambeth Palace Road, London SE1 7EH, UK. iheanyi.okpala@gstt.sthames.nhs.uk

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

14684149

Citation

Okpala, Iheanyi. "The Intriguing Contribution of White Blood Cells to Sickle Cell Disease - a Red Cell Disorder." Blood Reviews, vol. 18, no. 1, 2004, pp. 65-73.
Okpala I. The intriguing contribution of white blood cells to sickle cell disease - a red cell disorder. Blood Rev. 2004;18(1):65-73.
Okpala, I. (2004). The intriguing contribution of white blood cells to sickle cell disease - a red cell disorder. Blood Reviews, 18(1), pp. 65-73.
Okpala I. The Intriguing Contribution of White Blood Cells to Sickle Cell Disease - a Red Cell Disorder. Blood Rev. 2004;18(1):65-73. PubMed PMID: 14684149.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - The intriguing contribution of white blood cells to sickle cell disease - a red cell disorder. A1 - Okpala,Iheanyi, PY - 2003/12/20/pubmed PY - 2004/9/24/medline PY - 2003/12/20/entrez SP - 65 EP - 73 JF - Blood reviews JO - Blood Rev. VL - 18 IS - 1 N2 - Sickle cell disease (SCD) is characterized by a point mutation that replaces adenine with thymidine in the sixth codon of the beta-globin gene, a unique morphological abnormality of red blood cells, vaso-occlusion with ischaemic tissue injury, and susceptibility to infections. Vascular lumen obstruction in SCD results from interaction of erythrocytes, leukocytes, platelets, plasma proteins, and the vessel wall. The disease phenotype is a product of various genes and environmental factors acting in concert with the protein lesion underlying the red cell anomaly. The severity of SCD increases with leukocyte count. The biological basis and therapeutic implications of this relationship are discussed. Leukocytes contribute to SCD by adhering to blood vessel walls and obstructing the lumen, aggregating with other blood cells with more effective blockage of the lumen, stimulating the vascular endothelium to increase its expression of ligands for adhesion molecules on blood cells, and causing tissue damage and inflammatory reaction which predispose to vaso-occlusion. Patients with impaired ability of leukocytes to kill microbes are more prone to infections; which precipitate sickle cell crisis. Reduction of leukocyte count ameliorates SCD. Similarly, targeted blockade or reduced synthesis of specific leukocyte adhesion molecules and their ligands might confer clinical benefit in SCD. SN - 0268-960X UR - https://www.unboundmedicine.com/medline/citation/14684149/The_intriguing_contribution_of_white_blood_cells_to_sickle_cell_disease___a_red_cell_disorder_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0268960X03000377 DB - PRIME DP - Unbound Medicine ER -