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The percentage of dense red cells does not predict incidence of sickle cell painful crisis.

Abstract

To test the hypothesis that the tendency of hemoglobin S (HbS) to polymerize within cells is the major determinant of the incidence of vaso-occlusive episodes, we have examined the effect of the percentage of dense cells (as measured by Percoll-Stractan continuous density gradient centrifugation) on the frequency of painful crises in a group of 36 patients with sickle cell disease. No correlation was found between the percentage of dense cells and admissions for crisis. Among the patients with known alpha-gene status (n = 25), the strong correlation between decreased dense cells and alpha-thalassemia (-alpha/alpha alpha) reported previously was confirmed (P less than .001). In addition, in this small subset, patients with alpha-thalassemia (-alpha/alpha alpha) appeared to have a marginally increased number of admissions for sickle cell crisis (t = 2.1910, P less than .05), which was independent of the percentage of dense cells. We conclude that the percentage of dense sickle cells cannot predict the incidence of painful crisis, suggesting that other factors (microcirculatory regulation or other humoral and cellular factors) are more important in the generation maintenance of painful crises than the necessary, but not sufficient, tendency of HbS-containing red cells to sickle.

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  • Authors

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    Source

    Blood 68:1 1986 Jul pg 301-3

    MeSH

    Adult
    Anemia, Sickle Cell
    Erythrocyte Count
    Erythrocytes
    Female
    Globins
    Hemoglobin, Sickle
    Humans
    Male
    Middle Aged
    Prognosis

    Pub Type(s)

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

    Language

    eng

    PubMed ID

    3719102

    Citation

    Billett, H H., et al. "The Percentage of Dense Red Cells Does Not Predict Incidence of Sickle Cell Painful Crisis." Blood, vol. 68, no. 1, 1986, pp. 301-3.
    Billett HH, Kim K, Fabry ME, et al. The percentage of dense red cells does not predict incidence of sickle cell painful crisis. Blood. 1986;68(1):301-3.
    Billett, H. H., Kim, K., Fabry, M. E., & Nagel, R. L. (1986). The percentage of dense red cells does not predict incidence of sickle cell painful crisis. Blood, 68(1), pp. 301-3.
    Billett HH, et al. The Percentage of Dense Red Cells Does Not Predict Incidence of Sickle Cell Painful Crisis. Blood. 1986;68(1):301-3. PubMed PMID: 3719102.
    * Article titles in AMA citation format should be in sentence-case
    TY - JOUR T1 - The percentage of dense red cells does not predict incidence of sickle cell painful crisis. AU - Billett,H H, AU - Kim,K, AU - Fabry,M E, AU - Nagel,R L, PY - 1986/7/1/pubmed PY - 1986/7/1/medline PY - 1986/7/1/entrez SP - 301 EP - 3 JF - Blood JO - Blood VL - 68 IS - 1 N2 - To test the hypothesis that the tendency of hemoglobin S (HbS) to polymerize within cells is the major determinant of the incidence of vaso-occlusive episodes, we have examined the effect of the percentage of dense cells (as measured by Percoll-Stractan continuous density gradient centrifugation) on the frequency of painful crises in a group of 36 patients with sickle cell disease. No correlation was found between the percentage of dense cells and admissions for crisis. Among the patients with known alpha-gene status (n = 25), the strong correlation between decreased dense cells and alpha-thalassemia (-alpha/alpha alpha) reported previously was confirmed (P less than .001). In addition, in this small subset, patients with alpha-thalassemia (-alpha/alpha alpha) appeared to have a marginally increased number of admissions for sickle cell crisis (t = 2.1910, P less than .05), which was independent of the percentage of dense cells. We conclude that the percentage of dense sickle cells cannot predict the incidence of painful crisis, suggesting that other factors (microcirculatory regulation or other humoral and cellular factors) are more important in the generation maintenance of painful crises than the necessary, but not sufficient, tendency of HbS-containing red cells to sickle. SN - 0006-4971 UR - https://www.unboundmedicine.com/medline/citation/3719102/The_percentage_of_dense_red_cells_does_not_predict_incidence_of_sickle_cell_painful_crisis_ L2 - http://www.bloodjournal.org/cgi/pmidlookup?view=long&pmid=3719102 DB - PRIME DP - Unbound Medicine ER -