chapter 4

Question 1

step 1 of primary hemostasis (mediated by)

Answer 1

vasoconstiction

neural reflex, endothelin

Question 2

step 2 of primary hemostasis

Answer 2

platelet adhesion to VWF via GP1b

Question 3

step 3 of primary hemostasis

Answer 3

platelet degranulation- ADP and TXA2

Question 4

action of ADP

Answer 4

exposure of GP11a/IIIb on platelet surface

Question 5

action of TXA2

Answer 5

promotes aggregation

Question 6

step 4 of primary hemostasis

Answer 6

platelets aggregate to form plug

Question 7

2 types of disorders of primary hemostasis

Answer 7

quantitative- decreased # platelets

qualitative- impaired functioning of platelets

Question 8

clinical findings of TTP/HUS

Answer 8

skin/mucosal bleeding
microangiopathic HA (schistocytes)
fever
renal insuff- HUS
CNS abnormalities- TTP

Question 9

bernard-soulier

Answer 9

GPIb deficiency

Question 10

galnzmans

Answer 10

GPIIb/IIIa deficiency

Question 11

parosysmal nocturnal hematuria (cause)

Answer 11

due to defect in PIGA that leads to decreased GP1 on RBC surface = lack of CD55/59 which leaves RBCs susceptible to complement mediated lysis

Question 12

PHN clinical (4)

Answer 12

hemolytic anemia
pancytopenia
thrombosis
hemoglobinuria

Question 13

uremia and bleeding

Answer 13

impairs platelet function= leads to d/o of primary hemostasis

Question 14

complications of hereditary spherocytosis (2)

Answer 14

pigmented gallstones

aplastic crisis in parvo B19 infection

Question 15

factor V leiden

Answer 15

mutation (glutamine - arginine subs) in factor V makes it resistant to inactivation by activated protein C = increased thrombosis

Question 16

responsible for converting substances to carcinogens

Answer 16

microsomal monoxygenases (CYP 450)

Question 17

role of haptoglobin

Answer 17

binds free hemoglobin

Question 18

when is haptoglobin decreased

Answer 18

hemolysis

Question 19

acute intermittent porphyria enzyme defect

Answer 19

PBG deaminase

Question 20

acute intermittent porphyria symptoms

Answer 20

abdominal pain
neuro
port-wine urine
PBG and ALA in urine

Question 21

causes of pure red cell aplasia (3)

Answer 21

thymoma
lymphocytic leukemia
parvo B19 infection

Question 22

role of fibronectin

Answer 22

attaches cell integrin to collagen (anchors)

Question 23

cause of HUS (2)

Answer 23

E coli or shigella infection

Question 24

HUS symptoms (3)

Answer 24

hemolytic anemia with schistocytes
thrombocytopenia
acute kidney injury