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
Y1 - 1986
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 α-gene status (n=25), the strong correlation between decreased dense cells and α-thalassemia (-α/αα) reported previously was confirmed (P<.001). In addition, in this small subset, patients with α-thalassemia (-α/αα) appeared to have a marginally increased number of admissions for sickle cell crisis (t=2.1910, P<.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.
AB - 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 α-gene status (n=25), the strong correlation between decreased dense cells and α-thalassemia (-α/αα) reported previously was confirmed (P<.001). In addition, in this small subset, patients with α-thalassemia (-α/αα) appeared to have a marginally increased number of admissions for sickle cell crisis (t=2.1910, P<.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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U2 - 10.1182/blood.v68.1.301.301
DO - 10.1182/blood.v68.1.301.301
M3 - Article
C2 - 3719102
AN - SCOPUS:0022454177
SN - 0006-4971
VL - 68
SP - 301
EP - 303
JO - Blood
JF - Blood
IS - 1
ER -