The paradox of the serrated sickle erythrocyte: The importance of the red blood cell membrane topography

Ballas, Samir K.; Connes, Philippe

doi:10.3233/ch-152014

Cited by 6 publications

(4 citation statements)

References 13 publications

(6 reference statements)

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“…Concerning rs2070744 TT, T test -Mean ± standard deviation (n -sample size). 2 Mann-Whitney test -Median; minimum -maximum (n -sample size). results are not unexpected since the corresponding T allele in the promoter region was already reported associated with lower levels of gene expression [22].…”

Section: Discussionmentioning

confidence: 99%

“…Thus, T test -Mean ± standard deviation (n -sample size). 2 Mann-Whitney test -Median; minimum -maximum (n -sample size). Fig.…”

Section: Discussionmentioning

confidence: 99%

“…Hemolytic anemia and vaso-occlusion are the major features of this disease. Red blood cell rheology and adhesiveness play a key role in the occurrence of vaso-occlusive like events in sickle cell anemia, with stiffness of sickle erythrocytes and topography of cell membrane affecting adhesion to the endothelium [2].…”

Section: Introductionmentioning

confidence: 99%

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Sickle cell anemia – Nitric oxide related genetic modifiers of hematological and biochemical parameters

Aguiar

Matos

Gil

et al. 2017

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Abstract. BACKGROUND:Sickle cell anemia (SCA) is an inherited blood disorder. SCA patients present clinical and hematologic variability that cannot be only explained by the single mutation in the beta-globin gene. Others genetic modifiers and environmental effects are important for the clinical phenotype. SCA patients present arginine deficiency that contributes to a lower nitric oxide (NO) bioactivity. OBJECTIVE: The aim of this work is to determine the association between hematological and biochemical parameters and genetic variants from eNOS gene, in pediatric SCA patients. METHODS: 26 pediatric SCA patients were genotyped using polymerase chain reaction (PCR) and restriction fragment length polymorphism (RFLP) techniques in three important eNOS gene polymorphisms -rs2070744, rs1799983 and intron 4 VNTR. RESULTS: Results from this study show a significant statistical association between some parameters and genetic variants: an increased reticulocyte count and high serum lactate dehydrogenase levels were associated with both the rs2070744 TT and the rs1799983 GG genotypes at eNOS gene and high levels of neutrophils were associated with the eNOS4a allele at intron 4 VNTR. CONCLUSIONS: Our results reinforce the importance of NO bioactivity in SCA. We presume that NO, and its precursors might be used as therapy to improve the quality of life of SCA patients.

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Section: Discussionmentioning

confidence: 99%

“…Thus, T test -Mean ± standard deviation (n -sample size). 2 Mann-Whitney test -Median; minimum -maximum (n -sample size). Fig.…”

Section: Discussionmentioning

confidence: 99%

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Sickle cell anemia – Nitric oxide related genetic modifiers of hematological and biochemical parameters

Aguiar

Matos

Gil

et al. 2017

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“…On the other hand in extreme cases of sickle cell crisis, a dramatic near zero flow in cutaneous capillaries can occur [21,24] because of the extremely reduction of the deformability of the sickled erythrocytes [27] together with their adhesiveness [3].…”

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confidence: 99%

Role of blood viscosity in the microcirculation

Jung

Rampling

2017

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Blood fluidity is reported to influence capillary perfusion in healthy subjects and especially in patients with different diseases [6,10,29,31]. Up to now there is no simple way to quantify the influence of the different haemorheological parameters (haematocrit, plasma viscosity, erythrocyte aggregation, erythrocyte deformability) on the velocity of blood cells in human capillaries. In general, only a limited correlation can be shown [18]. One reason is the great variability of the diameter of human capillaries described in healthy subjects, with values between 3 and 15 m, and in patientsdepending on the disease -with values up to 50 m (e.g. giant capillaries in patients with scleroderma [33]). This is a very large range with respect to the role of the various haemorheological parameters of blood in microcirculation.The hypothesis of Barras, that plasma viscosity determines the perfusion of microvessels because the absolute capillary haematocrit is about 10-20%, so that blood viscosity in capillaries approaches plasma viscosity seems to be near the truth [4], as long as the capillary diameter is big in relation to the erythrocyte diameter. The work of Lipowsky would seem to confirm this [25]. In the case where the capillary diameter is smaller than the red blood cell diameter, the cells have to be deformed by shear forces before passing through a capillary. In this case the blood flow velocity should additionally depend on the erythrocyte deformability. This fact, which results from a fluid-dynamical point of view, however, is often not seen in patients using capillary microscopy in nailfold capillaries: neighbouring capillaries with different diameters and fed from the same arteriole show the same red blood cell velocity. Therefore we conclude that there is a regulatory mechanism in the capillary network possibly by the microvascular endothelium as described by different groups [8,15,26,34].In an animal model Driessen showed that rigidified erythrocytes slow down the capillary blood flow (up to stasis) only if the blood pressure was lowered below the normotensive state [9]. On the other hand in extreme cases of sickle cell crisis, a dramatic near zero flow in cutaneous capillaries can occur [21,24] because of the extremely reduction of the deformability of the sickled erythrocytes [27] together with their adhesiveness [3].The problem is more complicated in cases of a pathologically elevated erythrocyte aggregation. Under these conditions a massive sludging can be found -firstly described by M.H. Knisely´s group in conjunctival vessels -even in very small cutaneous capillaries [12]. Big erythrocyte aggregates pass very slowly through the capillary while immediately thereafter a single cell perfusion with significantly 1386-0291/16/$35.00

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Individual variability in response to a single sickling event for normal, sickle cell, and sickle trait erythrocytes

Tarasev

Muchnik

Light

et al. 2017

Translational Research

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The paradox of the serrated sickle erythrocyte: The importance of the red blood cell membrane topography

Cited by 6 publications

References 13 publications

Sickle cell anemia – Nitric oxide related genetic modifiers of hematological and biochemical parameters

Sickle cell anemia – Nitric oxide related genetic modifiers of hematological and biochemical parameters

Role of blood viscosity in the microcirculation

Individual variability in response to a single sickling event for normal, sickle cell, and sickle trait erythrocytes

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