Residual amounts of glycoproteins IIb and IIIa may be present in the
platelets of most patients with Glanzmann's thrombasthenia
AT Nurden, D Didry, N Kieffer and RP McEver
Glanzmann's thrombasthenia is an inherited bleeding disorder characterized
by abnormalities of platelet membrane glycoproteins (GP) IIb and IIIa. Most
patients, usually designated as type I, have been reported to have
undetectable levels of GP IIb and GP IIIa with the assay used. We have used
polyclonal rabbit antibodies against GP IIb and GP IIIa in a sensitive
immunoblot procedure capable of revealing trace amounts of these
glycoproteins. Platelets from nine thrombasthenic patients, including seven
with type I disease, were studied. GP IIIa, although decreased, was clearly
detectable in platelets of eight patients and GP IIb was identified in
five. Our findings suggest that residual quantities of GP IIb and GP IIIa
are present in most patients with thrombasthenia and therefore that major
deletions in the gene or genes encoding these proteins are uncommon.
Volume 65,
Issue 4,
pp. 1021-1024,
04/01/1985
Copyright © 1985 by The American Society of Hematology
