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Rheologic predictors of the severity of the painful sickle cell crisis.

Abstract

Deformable sickle erythrocytes have been reported by Mohandas and Evans to be more adherent to vascular endothelium than rigid irreversibly sickled cells (ISC). To define the clinical implications of this finding we have determined genetic, hematological, clinical, and rheological characteristics of sickle erythrocytes obtained from 65 patients with sickle cell anemia and fetal hemoglobin (Hb F) levels less than 15%. The alpha-globin gene number had a significant effect on the hematological parameters, the percentage of dense cells, ISC number, and HB A2 levels. The presence or absence of alpha thalassemia, however, had no effect on the frequency and severity of the sickle cell painful crisis (r = 0.06, P greater than .05). RBC deformability, determined by an ektacytometer, showed great heterogeneity among patients with three or four alpha-globin genes. Linear regression analyses of the data showed significant positive correlation of the frequency and severity of the painful crisis with RBC deformability (r = 0.49, P less than .001), and negative correlations with the percentage of dense cells (r = -0.37, P = .002), and the percentage of ISC (r = -0.46, P less than .001). We propose that the more deformable the sickle RBC are, the greater their adherence to vascular endothelium, and the more they cause vaso-occlusive crises, RBC deformability and the percentage of dense cells (or ISC) seem to have a predictive value of the frequency and severity of painful crises in sickle cell anemia.

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  • Authors+Show Affiliations

    ,

    Department of Medicine, Thomas Jefferson University, Philadelphia, PA.

    , , , ,

    Source

    Blood 72:4 1988 Oct pg 1216-23

    MeSH

    Adolescent
    Adult
    Anemia, Sickle Cell
    Child
    Erythrocyte Deformability
    Erythrocytes, Abnormal
    Female
    Genes
    Genotype
    Globins
    Humans
    Male
    Middle Aged
    Pain
    Regression Analysis
    Rheology

    Pub Type(s)

    Journal Article
    Research Support, Non-U.S. Gov't
    Research Support, U.S. Gov't, P.H.S.

    Language

    eng

    PubMed ID

    3167204

    Citation

    Ballas, S K., et al. "Rheologic Predictors of the Severity of the Painful Sickle Cell Crisis." Blood, vol. 72, no. 4, 1988, pp. 1216-23.
    Ballas SK, Larner J, Smith ED, et al. Rheologic predictors of the severity of the painful sickle cell crisis. Blood. 1988;72(4):1216-23.
    Ballas, S. K., Larner, J., Smith, E. D., Surrey, S., Schwartz, E., & Rappaport, E. F. (1988). Rheologic predictors of the severity of the painful sickle cell crisis. Blood, 72(4), pp. 1216-23.
    Ballas SK, et al. Rheologic Predictors of the Severity of the Painful Sickle Cell Crisis. Blood. 1988;72(4):1216-23. PubMed PMID: 3167204.
    * Article titles in AMA citation format should be in sentence-case
    TY - JOUR T1 - Rheologic predictors of the severity of the painful sickle cell crisis. AU - Ballas,S K, AU - Larner,J, AU - Smith,E D, AU - Surrey,S, AU - Schwartz,E, AU - Rappaport,E F, PY - 1988/10/1/pubmed PY - 1988/10/1/medline PY - 1988/10/1/entrez SP - 1216 EP - 23 JF - Blood JO - Blood VL - 72 IS - 4 N2 - Deformable sickle erythrocytes have been reported by Mohandas and Evans to be more adherent to vascular endothelium than rigid irreversibly sickled cells (ISC). To define the clinical implications of this finding we have determined genetic, hematological, clinical, and rheological characteristics of sickle erythrocytes obtained from 65 patients with sickle cell anemia and fetal hemoglobin (Hb F) levels less than 15%. The alpha-globin gene number had a significant effect on the hematological parameters, the percentage of dense cells, ISC number, and HB A2 levels. The presence or absence of alpha thalassemia, however, had no effect on the frequency and severity of the sickle cell painful crisis (r = 0.06, P greater than .05). RBC deformability, determined by an ektacytometer, showed great heterogeneity among patients with three or four alpha-globin genes. Linear regression analyses of the data showed significant positive correlation of the frequency and severity of the painful crisis with RBC deformability (r = 0.49, P less than .001), and negative correlations with the percentage of dense cells (r = -0.37, P = .002), and the percentage of ISC (r = -0.46, P less than .001). We propose that the more deformable the sickle RBC are, the greater their adherence to vascular endothelium, and the more they cause vaso-occlusive crises, RBC deformability and the percentage of dense cells (or ISC) seem to have a predictive value of the frequency and severity of painful crises in sickle cell anemia. SN - 0006-4971 UR - https://www.unboundmedicine.com/medline/citation/3167204/Rheologic_predictors_of_the_severity_of_the_painful_sickle_cell_crisis_ L2 - http://www.bloodjournal.org/cgi/pmidlookup?view=long&pmid=3167204 DB - PRIME DP - Unbound Medicine ER -