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Cellular and membrane properties of alpha and beta thalassemic erythrocytes
are different: implication for differences in clinical manifestations
SL Schrier, E Rachmilewitz and N Mohandas
Department of Medicine, Stanford University, CA.
To define how excess unpaired alpha- and beta-globin chains in severe
beta-thalassemia and severe alpha-thalassemia interacting with the membrane
might alter cellular and membrane properties, we performed a series of
biophysical and biochemical analyses on erythrocytes obtained from affected
patients. Detailed analysis of cellular and membrane deformability
characteristics showed that both forms of thalassemic erythrocytes have
excess surface area in relation to cell volume and increased membrane
dynamic rigidity. The deformability characteristics of thalassemic
erythrocytes in hypertonic media differed significantly from that of normal
erythrocytes of identical cell density. These findings suggest that dynamic
rigidity of thalassemic erythrocytes is influenced not only by cytoplasmic
viscosity determined by cell hemoglobin concentration but also by the
extent and type of globin interacting with the membrane. In contrast to the
above-noted similarities, major differences were noted in the mechanical
stability of the alpha- and beta-thalassemic membranes and in their state
of cell hydration. While the mechanical stability of alpha-thalassemic
membranes was normal or marginally elevated, the stability of beta-
thalassemic membranes was markedly decreased to half the normal value.
Cell-density analysis showed that the alpha-thalassemic erythrocytes were
uniformly less dense than normal, while beta-thalassemic erythrocytes had a
broad-density distribution, with all populations having both lower and
higher than normal density values, implying cellular dehydration in
beta-thalassemia and not in alpha-thalassemia. Membrane-protein analysis
revealed that excess globin chains were bound to the membrane skeletons of
both alpha- and beta-thalassemic erythrocytes, with the highest amounts
being found in membrane skeletons derived from erythrocytes of
splenectomized individuals with beta-thalassemia intermedia. These data
demonstrate that interaction of excess alpha- and beta-globin chains with
membranes produces different cellular changes and suggest that the observed
differences in the pathophysiology of alpha- and beta-thalassemias may be
related to different cellular effects induced by the excess in beta- and
alpha- globin chains.
Volume 74,
Issue 6,
pp. 2194-2202,
11/01/1989
Copyright © 1989 by The American Society of Hematology
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