Haematologica, Vol 94, Issue 8, 1060-1065 doi:10.3324/haematol.2008.005371
Copyright © 2009 by Ferrata Storti Foundation
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Red Cell Disorders

Red blood cell aggregation, aggregate strength and oxygen transport potential of blood are abnormal in both homozygous sickle cell anemia and sickle-hemoglobin C disease

Julien Tripette1,2, Tamas Alexy3, Marie-Dominique Hardy-Dessources2, Daniele Mougenel4, Eric Beltan5, Tawfik Chalabi5, Roger Chout5, Maryse Etienne-Julan4, Olivier Hue1, Herbert J. Meiselman3, Philippe Connes1

1 EA 3596, Laboratoire ACTES, Département de Physiologie, Université des Antilles et de la Guyane, Campus de Fouillole, Pointe-à-Pitre, Guadeloupe
2 UMR S 763 Inserm/Université des Antilles et de la Guyane, CHU Pointe-à-Pitre, Guadeloupe
3 Department of Physiology and Biophysics, University of Southern California, Keck School of Medicine, Los Angeles, CA, USA
4 Caribbean Sickle Cell Center, Pointe-à-Pitre, Guadeloupe
5 Department of Hematology/Immunology, Academic Hospital of Pointe-à-Pitre, Guadeloupe

Correspondence: Philippe Connes, PhD, Laboratoire ACTES (EA 3596), Département de Physiologie, Université des Antilles et de la Guyane, Campus de Fouillole, 97159 Pointe-à-Pitre, Guadeloupe (French West Indies). E-mail:pconnes{at}yahoo.fr

Background: Recent evidence suggests that red blood cell aggregation and the ratio of hematocrit to blood viscosity (HVR), an index of the oxygen transport potential of blood, might considerably modulate blood flow dynamics in the microcirculation. It thus seems likely that these factors could play a role in sickle cell disease.

Design and Methods: We compared red blood cell aggregation characteristics, blood viscosity and HVR at different shear rates between sickle cell anemia and sickle cell hemoglobin C disease (SCC) patients, sickle cell trait carriers (AS) and control individuals (AA).

Results: Blood viscosity determined at high shear rate was lower in sickle cell anemia (n=21) than in AA (n=52), AS (n=33) or SCC (n=21), and was markedly increased in both SCC and AS. Despite differences in blood viscosity, both sickle cell anemia and SCC had similar low HVR values compared to both AA and AS. Sickle cell anemia (n=21) and SCC (n=19) subjects had a lower red blood cell aggregation index and longer time for red blood cell aggregates formation than AA (n=16) and AS (n=15), and a 2 to 3 fold greater shear rate required to disperse red blood cell aggregates.

Conclusions: The low HVR levels found in sickle cell anemia and SCC indicates a comparable low oxygen transport potential of blood in both genotypes. Red blood cell aggregation properties are likely to be involved in the pathophysiology of sickle cell disease: the increased shear forces needed to disperse red blood cell aggregates may disturb blood flow, especially at the microcirculatory level, since red blood cell are only able to pass through narrow capillaries as single cells rather than as aggregates.

Key words: sickle cell disease, red blood cell aggregation, viscosity, red blood cell deformability.