Partial expression of GP Ib measured by flow cytometry in two patients with Bernard-Soulier syndrome
A Bunescu, T Lindahl, NO Solum, S Schulman… - Thrombosis research, 1994 - Elsevier
Bernard-Soulier syndrome is a rare, congenital bleeding disorder caused by absent or
defective GP Ib platelet membrane receptor for the von Willebrand factor (vWF). We studied
two brothers with moderate bleeding symptoms. Bleeding time was prolonged and ristocetin-
induced platelet aggregation was absent. Flow cytometric analysis showed that both boys
had a subnormal expression of GP Ib. One antibody used (AN51) was bound only to 30% of
the platelets and at a subnormal density. A second antibody (SZ2) also bound at a …
defective GP Ib platelet membrane receptor for the von Willebrand factor (vWF). We studied
two brothers with moderate bleeding symptoms. Bleeding time was prolonged and ristocetin-
induced platelet aggregation was absent. Flow cytometric analysis showed that both boys
had a subnormal expression of GP Ib. One antibody used (AN51) was bound only to 30% of
the platelets and at a subnormal density. A second antibody (SZ2) also bound at a …
