The Biophysics of the Variants of Sickle-Cell Disease

Griggs, Robert C.; Harris, J. W.

doi:10.1001/archinte.1956.00250210061005

Cited by 45 publications

(14 citation statements)

References 20 publications

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“…Upon re-deoxygenation of the same sample, sickling began to appear at approximately 60 mmHg, which is in agreement with previous reports [1,[24][25][26][27]. These sickled cells were PDSCs with pointy edges (Fig.…”

Section: Percentage Of Sickled Cells In the Blood Collected From A Pasupporting

confidence: 92%

Exposure of blood from patients with sickle cell disease to air changes the morphological, oxygen‐binding, and sickling properties of sickled erythrocytes

et al. 2005

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We collected venous blood samples from 7 steady-state patients with homozygous sickle cell disease under venous oxygen pressure without exposure to air (UnExpblood) and compared the morphological, oxygen-binding, and sickling properties with those of SS cells in aliquots of the same venous blood samples that were oxygenated in room air or at a PO 2 near 180 mmHg (Exp-blood). Results showed that (1) upon deoxygenation under nitrogen, UnExp-blood generated a significantly higher percentage of elongated reversibly sickled cells (RSCs) than did Exp-blood; (2) upon gradual oxygenation of completely deoxygenated sickled cells, RSCs in UnExp-blood converted to discocytes at a higher oxygen pressure than did those in Exp-blood; (3) the degree of hysteresis between the sickling/desickling curves of UnExp-blood was greater than that of Exp-blood; and (4) deoxy-Hb S in hemolysate prepared from SS cells in UnExp-blood polymerized without a delay time, while those from Exp-blood polymerized with a distinct delay time. The in vivo properties of RSCs significantly changed upon oxygenation. We also found that the various properties of blood samples collected from patients with SCD by the ordinary method were similar to those of Expblood, probably because such blood samples are exposed to oxygen through air in the needle, syringe, and Vacutainer. Once SS cells were oxygenated, the in vivo properties of RSCs could not be recovered by partial deoxygenation to venous oxygen pressure.

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Section: Percentage Of Sickled Cells In the Blood Collected From A Pasupporting

confidence: 92%

Exposure of blood from patients with sickle cell disease to air changes the morphological, oxygen‐binding, and sickling properties of sickled erythrocytes

et al. 2005

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show abstract

“…An increase of MCSHC to 11.9 grams per cent when the MCHC was 27 grams per cent was sufficient to permit sickling after complete dexoygenation; this effect was still greater when the MCHC reached 30 grams per cent and the MCSHC was 13.2 grams per cent. DISCUSSION The importance of changes in the viscosity of whole blood in vivo in determining much of the pathologic physiology of the sickle cell syndromes (2,3,8,20,21) is supported by the present data. The S hemoglobin concentration is evidently the chief determinant in bringing about the changes that are observed in vitro.…”

Section: Resultssupporting

confidence: 83%

Studies on the Destruction of Red Blood Cells. Xii. Factors Influencing the Role of S Hemoglobin in the Pathologic Physiology of Sickle Cell Anemia and Related Disorders 1

Greenberg¹,

Kass²,

Castle³

1957

J. Clin. Invest.

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“…In Hb SC disease the presence of Hb C in conjunction with Hb S has been shown to potentiate the sickling process (36). Erythrocytes with Hb SC sickled at both higher oxygen tensions and pH as compared with cells with Hb SA (37,38).…”

Section: Resultsmentioning

confidence: 99%

Hemoglobin CC disease: rheological properties of erythrocytes and abnormalities in cell water

Murphy¹

1968

J. Clin. Invest.

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The Biophysics of the Variants of Sickle-Cell Disease

Cited by 45 publications

References 20 publications

Exposure of blood from patients with sickle cell disease to air changes the morphological, oxygen‐binding, and sickling properties of sickled erythrocytes

Exposure of blood from patients with sickle cell disease to air changes the morphological, oxygen‐binding, and sickling properties of sickled erythrocytes

Studies on the Destruction of Red Blood Cells. Xii. Factors Influencing the Role of S Hemoglobin in the Pathologic Physiology of Sickle Cell Anemia and Related Disorders 1

Hemoglobin CC disease: rheological properties of erythrocytes and abnormalities in cell water

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