Bernard-Soulier syndrome involves deficiency of which receptor complex?

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Multiple Choice

Bernard-Soulier syndrome involves deficiency of which receptor complex?

Bernard-Soulier syndrome is caused by a deficiency of the GpIb-IX-V complex, the receptor on platelets that binds von Willebrand factor to mediate initial adhesion to the damaged vessel wall. When this complex is defective, platelets can’t effectively adhere, leading to mucocutaneous bleeding and the characteristic macrothrombocytopenia. This is why the correct receptor complex is the GpIb-IX-V cluster. In contrast, the GpIIb-IIIa complex is the fibrinogen receptor critical for platelet aggregation; its deficiency causes Glanzmann thromboasthenia, not Bernard-Soulier. The GpIa-IIa complex is another collagen receptor involved in platelet adhesion under certain conditions, but Bernard-Soulier specifically involves the GpIb-IX-V complex. The term GpIIa alone isn’t a standalone receptor complex, since it’s part of the GpIIb-IIIa receptor.

Bernard-Soulier syndrome is caused by a deficiency of the GpIb-IX-V complex, the receptor on platelets that binds von Willebrand factor to mediate initial adhesion to the damaged vessel wall. When this complex is defective, platelets can’t effectively adhere, leading to mucocutaneous bleeding and the characteristic macrothrombocytopenia. This is why the correct receptor complex is the GpIb-IX-V cluster.

In contrast, the GpIIb-IIIa complex is the fibrinogen receptor critical for platelet aggregation; its deficiency causes Glanzmann thromboasthenia, not Bernard-Soulier. The GpIa-IIa complex is another collagen receptor involved in platelet adhesion under certain conditions, but Bernard-Soulier specifically involves the GpIb-IX-V complex. The term GpIIa alone isn’t a standalone receptor complex, since it’s part of the GpIIb-IIIa receptor.

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