29. Which of the following is not true for bernard soulier syndrome ?
1. Autosomal dominant
2. Macrothrombocytopenia
3. Adhesion defect
4. GPIb-IX-V
Answer = [1] The Bernard-Soulier syndrome (BSS) and Glanzmann thrombasthenia (GT) are rare autosomal recessive disorders of platelet adhesion and aggregation, respectively, and present as mucocutaneous bleeding in infancy or childhood. The BSS is due to deficiency or dysfunction of the platelet membrane receptor for VWF, the GPIb-IX-V complex.
30. Which of the following is not true for gray platelet syndrome ?
1. Autosomal recessive
2. Mild to moderate bleeding
3. Microthrombocytopenia
4. Alpha granule deficiency
Answer = [3] The gray platelet syndrome is a rare autosomal recessive disorder due to platelet α-granule deficiency that causes mild to moderate bleeding, moderate macrothrombocytopenia, and platelets that have a gray appearance on Wright-stained blood smears.