Summary
In view of prior reports of platelet activation in the sickling disorders, platelet
aggregation, malondialdehyde (MDA) production following stimulation with N-ethylmaleimide,
and/or production time (survival) measurements were examined in 44 children with homozygous
sickle cell disease. Aggregation in response to epinephrine, collagen, and adenosine
diphosphate was similar to or only slightly less than in normal black controls, rendering
highly unlikely the circulation of a sizable population of refractory or “exhausted”
platelets. The platelets from the normal blacks aggregated less in response to epinephrine
than those from white control subjects. MDA generation in sickle cell platelets was
not increased, and platelet production time was not shortened in 6 patients studied
during crisis. These observations do not support the occurrence of a marked degree
of platelet activation and consumption in sickle cell anaemia.
Keywords
Sickle cell anaemia - Platelet aggregation - Malondialdehyde - Platelet activation
