Which of the following is a disorder with absent platelet ...

[Haya94]

Which of the following is a disorder with absent platelet glycoprotein Ib/IX/V and in which the patient has moderate thrombocytopenia, large platelets, and normal aggregometry with all agents except ristocetin?
 
  a. von Willebrand disorder (VWD)
  b. Bernard-Soulier syndrome
  c. Glanzmann thrombasthenia
  d. Dense granule deficiency

Question 2

What are the primary clinical manifestations of essential thrombocythemia?
 
  a. Platelet dysfunction
  b. Hemorrhage
  c. Thrombosis
  d. All of the above

[batool]

Answer to Question 1

ANS: B
Patients with Bernard-Soulier syndrome have deficient glycoprotein Ib/IX/V receptors on their surface. This is inherited as an autosomal recessive disorder; heterozygotes are normal or near normal, but homozygotes have (usually) a low platelet count, prolonged bleeding time, and enlarged platelets. Aggregation is normal with all agonists except ristocetin.

Answer to Question 2

ANS: D
Patients with essential thrombocythemia have platelet dysfunction and may either bleed or experience thrombosis.

[Haya94]

Excellent

[Sarahjh]

TYSM