22 - Path lab - Hereditary bleeding

Question 1

congenital platelet order that causes defective adhesion

Answer 1

Bernard Soulier Syndrome - lack GP1b

Question 2

congenital platelet order that causes defective aggregation

Answer 2

Glanzmann’s thrombasthenia - lack of GP2b-3a

Question 3

congenital platelet order that causes defective secretion

Answer 3

storage pool deficiencies - lack alpha granules and/or dense bodies

Question 4

Glanzmann’s thrombasthenia

Answer 4

AR inheritance
defect in GP2b-3a, defective platelet aggregation
higher incidence in arab populations
typical platelet problem symptoms
nl platelet count
assoc w/ leukocyte adhesion deficiency-III (LAD-III) syndrome

Question 5

tx of Glanzmann’s thrombasthenia

Answer 5

platelet transfusions, DDAVP, epsilon aminocaproic acid