Sickle cell anemia: Erythrokinetics, blood volumes, and a study of possible determinants of severity

Steinberg, Martin H.; Dreiling, Bernard J.

doi:10.1002/ajh.2830020103

Cited by 36 publications

(21 citation statements)

References 25 publications

(20 reference statements)

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“…The literature suggested that there is a decrease in the number of sickle cells during a painful episode [7][8][9]. Other investigators did not find an association between the number of sickle cells and clinical characteristics or differences between the steady-state and during the acute pain episode [10][11][12]. We decided to rexamine this finding in the current work for patients at steady state.…”

Section: Discussionmentioning

confidence: 59%

Quantification of Sickle Cells in the Peripheral Smear as a Marker of Disease Severity

Álvarez

Montague

Marin

et al. 2014

Fetal and Pediatric Pathology

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Blinded readers examined peripheral smears of 108 children with steady sickle cell (SC) disease and controls by counting ten 100× microscope fields and calculating percent of irreversible and reversible SC from total red cell population SC index (SCI). SCI was correlated to disease severity, and transfusion, hydroxyurea, or neither. Controls had a mean of 0.28% SC (range 0-0.64). Children with hemoglobin SS had a mean SCI of 5.12% ± 5.37 (range 0-30). SCI increased 0.33% with each increasing year (p <0.0001). Patients with SCI > 0.64 were 3.32 times as likely to experience clinical complications (p = 0.0124). Although blood transfusions and hydroxyurea decreased percent of SC, 72% treated patients had SCI >0.64, correlating with persistent sickling. This standardized method quantifies SC in peripheral smears. Percent of SC increased with age and correlated with disease severity, especially hemolytic complications, providing readily available information with minimal or no extra cost.

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Section: Discussionmentioning

confidence: 59%

Quantification of Sickle Cells in the Peripheral Smear as a Marker of Disease Severity

Álvarez

Montague

Marin

et al. 2014

Fetal and Pediatric Pathology

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“…The similarity of the effects of fraction III and fraction IV on the hemodynamic characteristics studied might partly explain the persistent controversy that exists (15,16) over the contribution of morphologically defined ISC in the clinical severity of the disease.…”

Section: Hemodynamic Observationsmentioning

confidence: 93%

Erythrocytes in sickle cell anemia are heterogeneous in their rheological and hemodynamic characteristics.

Kaul¹,

Fabry²,

Windisch³

et al. 1983

J. Clin. Invest.

216

155

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A B S T R A C T To understand the contribution to the pathophysiology of sickle cell anemia of the different erythrocyte density types present in the blood of these patients, we have studied the viscosimetric and hemodynamic characteristics of four major classes of hemoglobin SS erythrocytes. We have isolated reticulocytes, discocytes, dense discocytes, and irreversibly sickled cells (fractions I-IV) on Percoll-Renografin density gradients. Bulk viscosity was studied in a coneplate viscosimeter and the hemodynamic studies were performed on the isolated, artificially perfused mesoappendix vasculature of the rat (Baez preparation).Bulk viscosity measurements at shear rates of 230 s-' demonstrate that when the cells are oxygenated, fraction I (reticulocyte rich) has a higher viscosity than expected from its low intracellular hemoglobin concentration. The rest of the fractions exhibit moderate increases in bulk viscosity pari-passu with the corresponding increases in density (mean corpuscular hemoglobin concentration). When deoxygenated, all cell fractions nearly doubled their bulk viscosity and the deoxy-oxy differences remained constant. The Baez preparation renders a different picture: oxygenated fractions behave as predicted by the viscosimetric data, but, when deoxygenated, cell fractions exhibit dramatically increased peripheral resistance and the This work was presented, in part, at the 66th Annual

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“…Although it has not been possible to demonstrate a direct correlation between the proportion of ISCs in the blood of a given patient and the frequency or severity of painful crises (1,2), evidence exists to support the idea that ISCs may be clinically important. First, Serjeant et al (3) have shown a direct relationship between the percentage of circulating ISCs and shortened erythrocyte survival.…”

Section: Introductionmentioning

confidence: 99%

Monovalent cation transport in irreversibly sickled cells.

Clark¹,

Morrison²,

Shohet³

1978

J. Clin. Invest.

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Sickle cell anemia: Erythrokinetics, blood volumes, and a study of possible determinants of severity

Cited by 36 publications

References 25 publications

Quantification of Sickle Cells in the Peripheral Smear as a Marker of Disease Severity

Quantification of Sickle Cells in the Peripheral Smear as a Marker of Disease Severity

Erythrocytes in sickle cell anemia are heterogeneous in their rheological and hemodynamic characteristics.

Monovalent cation transport in irreversibly sickled cells.

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