Chapter 5-7

Question 1

paroxysmal nocturnal hemoglobinuria

Answer 1

defect in myeloid stem cells absent glycosphosphatidylinositol (GPI- connects DAF and MIRL-proctect rbcs from compliment)

Question 2

physiology in paroxysmal nocturnal hemoglobinuria

Answer 2

at night shallow breathing we are acidotic activation of compliment

Question 3

screening test for paroxysmal nocturnal hemoglobinuria

Answer 3

sucrose test is used to screen for dz

Question 4

diagnositic test for paroxysmal nocturnal hemoglobinuria

Answer 4

acidified serum test or flow cytometry detcts lack of CD55 also called DAF

Question 5

main cause of death in paroxysmal nocturnal hemoglobinuria

Answer 5

thrombosis of hepatic portal or cerebral veins destroy plts

Question 6

paroxysmal nocturnal hemoglobinuria complications

Answer 6

AML and Fe def anemia

Question 7

Glucose 6 phosphate dehydrogenase

Answer 7

X linked reduced half life of G6PD renders cells susceptible to oxidative stress. REDUCED GLUTATHIONE leads to oxidative injury

Question 8

African variant G6PD

Answer 8

mildly reduced 1/2 life of G6PD leads to mild intravascular hemolysis with oxidative stress

Question 9

Mediterranean variant

Answer 9

more than 1/2 reduced 1/2 life of G6PD marked intravascular hemolysis with oxidative stress

Question 10

G6PD def protects against

Answer 10

falciparum maaria

Question 11

cells seen in G6PD

Answer 11

Heinz body