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OS Platt
We describe a steady-state patient with sickle cell anemia (SS disease) who
developed sporadic hemoglobinuria, historically related to vigorous
exercise. We studied him and four other patients with SS disease and
demonstrated exercise-induced hemoglobinemia. To see if SS erythrocytes
were abnormally fragile when exposed to shear forces that could be
generated in small vessels of exercising muscles, we exposed them to
physiologic shear rates in a cone-plate viscometer. We show that SS
erythrocytes are more shear sensitive than normal erythrocytes. This
phenomenon is directly related to the presence of dehydrated cells as
demonstrated by the increasing shear sensitivity of increasingly dehydrated
cells separated on Stractan density gradients. Normal shear sensitivity
could be restored to dehydrated layers by restoring normal hydration.
Restoration of shear stability was complete in all layers except for the
most dense ISC layer. A control group of patients with SC disease exhibited
no exercise-induced hemoglobinemia, no abnormal shear sensitivity of whole
blood, and only rare dehydrated ISCs. These studies suggest that the
exercise-induced hemolysis in SS patients is related to the lysis of
dehydrated, shear-sensitive cells. This same process may also contribute to
the chronic hemolysis of SS disease--a phenomenon known to correlate with
the numbers of dehydrated ISCs.
This article has been cited by other articles:
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| Copyright © 1982 by American Society of Hematology Online ISSN: 1528-0020 | |||||||||
