Blood Disorders

Question 1

von Willebrand Disease

Answer 1

Platelet defect: Defective or deficient VWF
Function affected: Adhesion(to vWF)
Primary aggregation (GPIIb/IIa-vWF)
Factor VIII (half-life prolonged by vWF)
Prolonged bleeding time and maybe aPPT (VIII)

Question 2

Bernard-Soulier Syndrome

Answer 2

Giant platelet syndrome
Platelet defect: defect or deficiency of GPIb protein
Platelet function affected: Adhesion (to vWF)

Question 3

Idiopathic Thrombocytopenic purpura (ITP)

Answer 3

Platelet defect: Immune response response against GPIIb/IIIa results in platelet destruction
Function affected: Platelets are destroyed by cells of immune system—> Thrombocytopenia

Question 4

Glanzmann Thombasthenia

Answer 4

Platelet defect: defect or deficiency of GPIIb/IIIa protein
Function affected: Primary aggregation ( GPIIb/IIIa-vWF)
Secondary aggregation (GPIIb/IIIa-fibrinogen)

Question 5

Thrombotic thrombocytopenic purpura (TTP)

Answer 5

Platelet defect: mutation and defective function of ADAMTS13 results in increased levels of high molecular weight vWF
Function: exaggerated thrombosis (increased platelet plug formation); thrombocytopenia

Question 6

DIC-Disseminated intravascular coagulation

Answer 6

Results in multiple microthrombi that unnecessarily uses up both platelet and coagulation factors

Question 7

Hemophilia A, B, or C

Answer 7

Factor affected: A (VIII), B (IX), C (XI)

Prolonged aPPT