The abnormal shape and poor deformability of the sickled erythrocyte (RBC) have generally been held responsible for the microvascular occlusions of sickle cell disease. However, there is no correlation between the clinical severity of this disease and the presence of sickled RBC. In searching for additional factors that might contribute to the pathophysiology of sickle cell disease, we have investigated the possibility that sickle RBC might be less than normally repulsive of the vascular endothelium. After RBC suspensions are allowed to settle onto plates of cultured human endothelial cells, normal RBC are completely removed by as few as six washes. In contrast, sickle RBC remain adherent despite multiple washes. On subconfluent culture plates, normal RBC are distributed randomly, whereas sickle RBC cluster around endothelial cells. Sickle RBC adherence is not enhanced by deoxygenation but does increase with increasing RBC density. The enzymatic removal of membrane sialic acid greatly diminishes the adherence of sickle RBC to endothelial cells, suggesting that sialic acid participates in this abnormal cell-cell interaction. Although net negative charge appears normal, sickle RBC mainfest an abnormal clumping of negative surface charge as demonstrated by localization of cationized ferritin. These abnormalities are reproduced in normal RBC loaded with nonechinocytogenic amounts of calcium. We conclude that sickle RBC adhere to vascular endothelial cells in vitro, perhaps caused by a calcium-induced aberration of membrane topography. This adherence may be a pathogenetic factor in the microvascular occlusions characteristic of sickle cell disease.
Robert P. Hebbel, Osamu Yamada, Charles F. Moldow, Harry S. Jacob, James G. White, John W. Eaton
Year: | 2025 | 2024 | 2023 | 2022 | 2021 | 2020 | 2019 | 2018 | 2017 | 2016 | 2015 | 2014 | 2013 | 2012 | 2011 | 2010 | 2009 | 2008 | 2007 | 2006 | 2005 | 2004 | 2003 | 2002 | 2001 | 2000 | 1999 | 1998 | 1997 | 1996 | 1995 | 1993 | 1992 | 1991 | 1990 | 1989 | 1988 | 1987 | 1986 | 1985 | 1984 | 1983 | 1982 | 1981 | 1980 | 1978 | 1952 | 1949 | Total |
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Citations: | 1 | 2 | 4 | 5 | 3 | 7 | 7 | 10 | 7 | 15 | 7 | 10 | 2 | 5 | 4 | 10 | 8 | 9 | 5 | 5 | 3 | 5 | 7 | 7 | 7 | 8 | 6 | 6 | 7 | 6 | 2 | 6 | 7 | 4 | 7 | 7 | 2 | 13 | 10 | 16 | 7 | 6 | 4 | 6 | 1 | 1 | 1 | 1 | 289 |
Title and authors | Publication | Year |
---|---|---|
Sickle cell disease biochip: a functional red blood cell adhesion assay for monitoring sickle cell disease
Y Alapan, C Kim, A Adhikari, KE Gray, E Gurkan-Cavusoglu, JA Little, UA Gurkan |
Translational Research | 2016 |
VLA-4 blockade by natalizumab inhibits sickle reticulocyte and leucocyte adhesion during simulated blood flow
J White, S Krishnamoorthy, D Gupta, M Lancelot, N Moore, S Sarnaik, WE Hobbs, DR Light, P Hines |
British Journal of Haematology | 2016 |
Sevuparin binds to multiple adhesive ligands and reduces sickle red blood cell-induced vaso-occlusion
MJ Telen, M Batchvarova, S Shan, PH Bovee-Geurts, R Zennadi, A Leitgeb, R Brock, M Lindgren |
British Journal of Haematology | 2016 |
INCREASED VASOOCCLUSIVE CRISIS IN “O” BLOOD GROUP SICKLE CELL DISEASE PATIENTS: ASSOCIATION WITH UNDERLYING THROMBOSPONDIN LEVELS
MA Huneini, A Pathare |
Mediterranean journal of hematology and infectious diseases | 2016 |
Crises in Sickle Cell Disease
EM Novelli, MT Gladwin |
Chest | 2016 |
Interactions of sickle red blood cells with neutrophils are stabilized on endothelial cell layers
VM Dominical, DM Vital, VT Garrido, AA Silveira, ST Olalla-Saad, FF Costa, N Conran |
Blood Cells, Molecules, and Diseases | 2016 |
Red blood cells in retinal vascular disorders
R Agrawal, J Sherwood, J Chhablani, A Ricchariya, S Kim, PH Jones, S Balabani, D Shima |
Blood Cells, Molecules, and Diseases | 2016 |
In Vivo T1 of Blood Measurements in Children with Sickle Cell Disease Improve Cerebral Blood Flow Quantification from Arterial Spin-Labeling MRI
L Vaclavu, V der Land, DF Heijtel, MJ van Osch, MH Cnossen, CB Majoie, A Bush, JC Wood, KJ Fijnvandraat, HJ Mutsaerts, AJ Nederveen |
American Journal of Neuroradiology | 2016 |
Effects of Poloxamer 188 on red blood cell membrane properties in sickle cell anaemia
B Sandor, M Marin, C Lapoumeroulie, M Rabaï, SD Lefevre, N Lemonne, WE Nemer, A Mozar, O Français, BL Pioufle, P Connes, CL van Kim |
British Journal of Haematology | 2016 |
Sickle cell disease: challenges and progress
LV Parise, N Berliner |
Blood | 2016 |
Micro- and Nanosystems for Biotechnology: Love/Micro- and Nanosystems for Biotechnology
ME Brett, DK Wood |
Micro- and Nanosystems for Biotechnology: Love/Micro- and Nanosystems for Biotechnology | 2016 |
Mobility Enhancement of Red Blood Cells with Biopolymers
D Tahara, N Oikawa, R Kurita |
Journal of the Physical Society of Japan | 2016 |
Modulation of Sickle Red Blood Cell Adhesion and its Associated Changes in Biomarkers by Sulfated Nonanticoagulant Heparin Derivative
A Alshaiban, V Muralidharan-Chari, A Nepo, SA Mousa |
Clinical and Applied Thrombosis/Hemostasis | 2016 |
Sickle Cell Disease
S Azar, TE Wong |
Medical Clinics of North America | 2016 |
Exacerbation of oxidative stress during sickle vaso-occlusive crisis is associated with decreased anti-band 3 autoantibodies rate and increased red blood cell-derived microparticle level: a prospective study
R Hierso, N Lemonne, R Villaescusa, ML Lalanne-Mistrih, K Charlot, M Etienne-Julan, B Tressières, Y Lamarre, V Tarer, Y Garnier, AA Hernandez, S Ferracci, P Connes, M Romana, MD Hardy-Dessources |
British Journal of Haematology | 2016 |