chapter 4-1

Question 1

thrombotic thrombocytopenic purpura

Answer 1

defect in ADAMTS13 E cleaves vWF multimer into smaller monomers. Defect leads to microthrombi- ADULT FEMALES- CNS thrombi. TX- corticosteroids
shistocyte-

Question 2

Hemolytic Uremic Syndrome

Answer 2

E.Coli O157/H7 verotoxin damages ADAMTS13 get PLT microthrombi- renal insuf.
-shistocyte

Question 3

Clinical findings of HUS & TTP

Answer 3

skin/mucosal bleeding, microangiopathic hemolytic anemia, fever

Question 4

Lab findings of HUS & TPP

Answer 4

Thrombocytopenia w/ increase Bleeding time
normal PT/PTT
increase megakaryocytes on bone marrow biopsy
increase in LDH

Question 5

Bernard Soulier Syndrome

Answer 5

genetic GPIb def.-plt adhesion is impaired bc GP1B cant adhere plt to endothelium
(big sucksers- enlarged plts on lab)

Question 6

Glanzmann thrombasthenia

Answer 6

genetic def in GP2B/3A def plt aggregation- plt cant bind together

Question 7

asprin

Answer 7

irreversibly inactivates COX- lack of TXA2 impairs aggregation