What is the role of adhesion molecules in the pathogenesis of sickle cell disease (SCD)?

Updated: May 12, 2021
  • Author: Joseph E Maakaron, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Answer

Several studies have shown involvement of an array of adhesion molecules expressed on sickle RBCs, including CD36, a-4-ß-1 integrin, intercellular cell adhesion molecule–4 (ICAM-4), and basal cell adhesion molecule (B-CAM). [10] Adhesion molecules (ie, P-selectin, VCAM-1, a-V-ß-3 integrin) are also expressed on activated endothelium. Finally, plasma factors and adhesive proteins (ie, thrombospondin [TSP], von Willebrand factor [vWf], laminin) play an important role in this interaction.

For example, the induction of VCAM-1 and P-selectin on activated endothelium is known to enhance sickle RBC interactions. In addition, a-V-ß-3 integrin is upregulated in activated endothelium in patients with sickle cell disease. a-V-ß-3 integrin binds to several adhesive proteins (TSP, vWf, red-cell ICAM-4, and, possibly, soluble laminin) involved in sickle RBC adhesion, and antibodies to this integrin dramatically inhibit sickle RBC adhesion.


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