Red cell life span in sickle cell-hemoglobin C disease with a note about
sickle cell-hemoglobin O ARAB
PR McCurdy, L Mahmood and AS Sherman
Red cell survival was measured in ten subjects with S-C disease and one
with S-O Arab (alpha 2 beta 2-121 glu yields lys) disease using both DF32p
and 51Cr as tags. Red cell volume was slightly reduced in most patients
(87% plus or minus 20% of predicted normal). In nine SC patients, mean red
cell life (DF32p) was 28.9 plus or minus 4.0 days. For one SC subject it
was significantly longer (47.9 days), as it was for the one with S-O Arab.
The S-O Arab subject had irreversibly sickled cells in the peripheral
blood, shereas those with SC had few (less than 1/1000 red cells) or none.
The S-O Arab hemolysate gelled at a hemmoglobin concentration (16.2
g/100ml) near that for sickle cell anemia hemolysates (15.9 plus or minus
1.0 g/100 ml; n equals 8) but significantly lower than that for SC
hemolysates (21.6 plus or minus 1.9 g/100 ml; n equals 5). It seems likely
that properties of S-C red cells other than their relative ease of sickling
contribute significantly to their rate of hemolysis.
Volume 45,
Issue 2,
pp. 273-279,
02/01/1975
Copyright © 1975 by The American Society of Hematology
