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Red cell adhesion in human diseases

Colin, Yvesa,b,c,d; Van Kim, Caroline Lea,b,c,d; El Nemer, Wassima,b,c,d

Current Opinion in Hematology: May 2014 - Volume 21 - Issue 3 - p 186–192
doi: 10.1097/MOH.0000000000000036
ERYTHROID SYSTEM AND ITS DISEASES: Edited by Narla Mohandas

Purpose of review This review discusses the unexpected role of red blood cell (RBC) adhesiveness in the pathophysiology of two red cell diseases, hereditary spherocytosis and polycythemia vera, and two ‘nonerythroid’ disorders, central retinal vein occlusion and Gaucher disease. These pathologies share common clinical manifestations, that is vaso-occlusion and/or thrombotic events.

Recent findings Recently, the direct involvement of RBC adhesion to the vascular endothelium has been demonstrated in the occurrence of vaso-occlusive events, in particular in sickle cell disease (SCD). Several erythroid adhesion molecules and their ligands have been identified that belong to different molecular classes (integrins, Ig-like molecules, lipids…) and are activated by a variety of signaling pathways. Among these, the laminin receptor, Lutheran/basal cell adhesion molecule, which is activated by phosphorylation, appears to play a central role in several pathologies.

Summary RBC adhesiveness might be involved in complications such as the vaso-occlusive crisis in SCD, thrombosis in polycythemia vera, splenic sequestration in hereditary spherocytosis, occlusions in central retinal vein occlusion and bone infarcts in Gaucher disease. Characterization of this pathological process at the cellular and molecular levels should prove useful to develop new therapeutic approaches based on the blockade of RBC abnormal interactions with vascular endothelium and/or circulating blood cells.

aInserm U1134

bUniversité Paris Diderot, Sorbonne Paris Cité, UMR_S 1134

cInstitut National de la Transfusion Sanguine

dLaboratoire d’ Excellence GR-Ex, Paris, France

Correspondence to Yves Colin, UMR_S 1134, INTS, 6 Rue Alexandre Cabanel, 75015 Paris, France. Tel: +00 33 1 44 49 30 93; e-mail: yves.colin-aronovicz@inserm.fr

© 2014 Wolters Kluwer Health | Lippincott Williams & Wilkins