Measurements of red cell deformability and hydration reflect HbF and HbA 2 in blood from patients with sickle cell anemia

Parrow, Nermi L.; Tu, Hongbin; Nichols, James S.; Violet, Pierre-Christian; Pittman, Corinne A.; Fitzhugh, Courtney D.; Fleming, Robert E.; Mohandas, Narla; Tisdale, John F.; Levine, Mark

doi:10.1016/j.bcmd.2017.04.005

Cited by 22 publications

(28 citation statements)

References 41 publications

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“…Our HU+ patients had higher RBC deformability than HU-individuals. This is in agreement with several previous studies showing that HU treatment improves RBC deformability in SCA, which participates to the clinical benefits observed with this drug [40][41][42][43][44]. The improvement of RBC deformability in HU treated patients has been mainly attributed to the increased production of HbF and a better RBC hydration status (higher mean cellular volume).…”

Section: Discussionsupporting

confidence: 92%

Hydroxyurea therapy modulates sickle cell anemia red blood cell physiology: Impact on RBC deformability, oxidative stress, nitrite levels and nitric oxide synthase signalling pathway

et al. 2018

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HAL is a multidisciplinary open access archive for the deposit and dissemination of scientific research documents, whether they are published or not. The documents may come from teaching and research institutions in France or abroad, or from public or private research centers. L'archive ouverte pluridisciplinaire HAL, est destinée au dépôt et à la diffusion de documents scientifiques de niveau recherche, publiés ou non, émanant des établissements d'enseignement et de recherche français ou étrangers, des laboratoires publics ou privés.

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Section: Discussionsupporting

confidence: 92%

Hydroxyurea therapy modulates sickle cell anemia red blood cell physiology: Impact on RBC deformability, oxidative stress, nitrite levels and nitric oxide synthase signalling pathway

et al. 2018

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“…Osmotic gradient ektacytometry, in which deformability is measured as a function of a continuous change in osmolarity, is a well‐established method, in particular in hereditary disorders of the red cell membrane . In SCD, osmotic gradient ektacytometry showed that increasing HbF induces a better surface‐to‐volume ratio thereby improving deformability in untransfused patients with SCD . RBC deformability during normoxic conditions as a marker for complications has also been investigated in the past, and a negative correlation was found between an increase in RBC deformability and osteonecrosis or painful sickle cell crises .…”

Section: Introductionmentioning

confidence: 99%

Rapid and reproducible characterization of sickling during automated deoxygenation in sickle cell disease patients

et al. 2019

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In sickle cell disease (SCD), sickle hemoglobin (HbS) polymerizes upon deoxygenation, resulting in sickling of red blood cells (RBCs). These sickled RBCs have strongly reduced deformability, leading to vaso‐occlusive crises and chronic hemolytic anemia. To date, there are no reliable laboratory parameters or assays capable of predicting disease severity or monitoring treatment effects. We here report on the oxygenscan, a newly developed method to measure RBC deformability (expressed as Elongation Index ‐ EI) as a function of pO2. Upon a standardized, 22 minute, automated cycle of deoxygenation (pO2 median 16 mmHg ± 0.17) and reoxygenation, a number of clinically relevant parameters are produced in a highly reproducible manner (coefficients of variation <5%). In particular, physiological modulators of oxygen affinity, such as, pH and 2,3‐diphosphoglycerate showed a significant correlation (respectively R = ‑0.993 and R = 0.980) with Point of Sickling (PoS5%), which is defined as the pO2 where a 5% decrease in EI is observed during deoxygenation. Furthermore, in vitro treatment with antisickling agents, including GBT440, which alter the oxygen affinity of hemoglobin, caused a reproducible left‐shift of the PoS, indicating improved deformability at lower oxygen tensions. When RBCs from 21 SCD patients were analyzed, we observed a significantly higher PoS in untreated homozygous SCD patients compared to treated patients and other genotypes. We conclude that the oxygenscan is a state‐of‐the‐art technique that allows for rapid analysis of sickling behavior in SCD patients. The method is promising for personalized treatment, development of new treatment strategies and could have potential in prediction of complications.

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“…Исследование производили при стабилизированной температуре 25 0 С. [6][7][8], при стомацитарном эллиптоцитозе и овалоцитозе [9,10], при обработке эритроцитов возрастающими концентрациями лизолецитина и диамида [11], гидроксимочевиной [12], динитробензолом [7], ацитретином [13]. Появлению двух гипотонических минимумов на осмосканах либо вовсе не уделяется внимания, либо объясняется присутствием клеточных популяций с 2-мя различными отношениями S/V, либо наличием вовсе недеформируемых клеток.…”

Section: рисунок 1 осмоскан эритроцитов здоровых доноров в нативной unclassified

On the Violation of the Rheological Properties of Erythrocytes During Biochemical Procedures

Katiukhin¹

2018

Проблемы И Вопросы Современной Науки.

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АННОТАЦИЯ Представлены изменения осмотических профилей деформируемости эритроцитов при инкубации с буфером HEPES и концентрационных нагрузках цельной крови ацетилхолином и трет-бутилгидропероксидом in vitro. Показано резкое снижение деформируемости в зоне 200-230 мосмоль/кг H 2 O. Феномен проявляется в виде выраженного излома профилей осмотической деформируемости. Дано объяснение найденным изменениям деформационных свойств эритроцитов. Ключевые слова: эритроцит, эктацитометрия, деформируемость.

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Measurements of red cell deformability and hydration reflect HbF and HbA 2 in blood from patients with sickle cell anemia

Cited by 22 publications

References 41 publications

Hydroxyurea therapy modulates sickle cell anemia red blood cell physiology: Impact on RBC deformability, oxidative stress, nitrite levels and nitric oxide synthase signalling pathway

Hydroxyurea therapy modulates sickle cell anemia red blood cell physiology: Impact on RBC deformability, oxidative stress, nitrite levels and nitric oxide synthase signalling pathway

Rapid and reproducible characterization of sickling during automated deoxygenation in sickle cell disease patients

On the Violation of the Rheological Properties of Erythrocytes During Biochemical Procedures

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