Inappropriately low red cell 2,3-diphosphoglycerate and p50 in transfused
beta-thalassemia
A Correra, JH Graziano, C Seaman and S Piomelli
The relationships among hemoglobin concentration (Hb), red cell 2,3-
diphosphoglycerate (2,3-DPG), and p50 were studied in 20 chronically
hypertransfused patients with thalassemia major. In the nontransfused
control group, which included normal individuals as well as patients with
sickle cell disease or iron deficiency anemia, the Hb correlated inversely
with both 2,3-DPG concentration and p50, as is well established. In
contrast, however, prior to transfusion, at the nadir of Hb, patients with
thalassemia major had inappropriately low 2,3-DPG concentrations and p50s.
These findings occurred in all patients, regardless of whether they had
received packed, leukocyte-poor, or frozen-thawed red cells. The hypothesis
that the time of blood storage was a factor was excluded by repeatedly
transfusing one patient with packed red cells administered within 4 hr of
collection in CPDA-1. A second hypothesis, that red cell function might be
impaired by the iron- overloaded thalassemic environment, was excluded by
studying a newly diagnosed, newly transfused patient with aplastic anemia.
In both cases, the same inability to appropriately increase 2,3-DPG and p50
as the Hb fell during the intertransfusion interval was noticed. These data
suggest that red cells of chronically transfused patients are unable to
adapt to the decline in Hb that occurs during the intertransfusion
interval.
Volume 63,
Issue 4,
pp. 803-806,
04/01/1984
Copyright © 1984 by The American Society of Hematology
