5.6 - Normocytic anemia with intravascular hemolysis

Question 1

paroxysmal nocturnal hemoglobinuria is an acquired defect in ______ stem cells resulting in absent _______, rendering cells susceptible to destruction by _______________

Answer 1

• myeloid
• GPI anchor
• complement

Question 2

where is decay accelerating factor located? how does it protect RBCs from complement mediated damage?

Answer 2

• surface of RBCs

- inhibition of C3 convertase

Question 3

what is the CD designation for DAF?

Answer 3

CD55

Question 4

what is the main cause of death in PNH?

Answer 4

thrombosis of hepatic, portal, or cerebral VEINS

Question 5

what is the inheritance of G6PD deficiency?

Answer 5

XR

Question 6

G6PD deficiency confers protection against plasmodium ____________

Answer 6

falciparum

Question 7

which is more serious - African or Mediterranean variant of G6PD deficiency?

Answer 7

Mediterranean

Question 8

what enzyme cannot be made in G6PD deficiency? what is the end result?

Answer 8

• NADPH

- glutathione cannot be reduced to prevent oxidative injury

Question 9

in G6PD deficiency oxidative stress precipitates Hb as ____________

Answer 9

heinz bodies

Question 10

heinz bodies are removed from RBCs by splenic macrophages, resulting in ___________

Answer 10

bite cells

Question 11

the hemolysis in G6PD deficiency is primarily _______________ (extravascular / intravascular)

Answer 11

intravascular

Question 12

why is there back pain in G6PD deficiency?

Answer 12

Hb is nephrotoxic

Question 13

what is the screening test for G6PD deficiency?

Answer 13

heinz preparation to look for precipitated Hb

Question 14

immune hemolytic anemia is mediated by either Ig__ or Ig__ destruction of RBCs?

Answer 14

IgG or IgM

Question 15

IgG mediated hemolytic anemia usually involves ___________ (intravascular / extravascular) hemolysis

Answer 15

extravascular

Question 16

is IgG associated with warm or cold agglutinins?

Answer 16

warm

Question 17

IgG mediated hemolytic anemia usually results in what type of RBC variant?

Answer 17

spherocyte

Question 18

a-methyldopa can cause what type of immune hemolytic anemia?

Answer 18

IgG mediated (warm agglutinin)

Question 19

IgM mediated hemolytic anemia usually involves ______________ (intravascular / extravascular) hemolysis

Answer 19

extravascular

Question 20

is IgM associated with warm or cold agglutinins?

Answer 20

cold

Question 21

which type of agglutinin is associated with SLE?

Answer 21

IgG (warm)

Question 22

which type of agglutinin is associated with mycoplasma?

Answer 22

IgM (cold)

Question 23

what process ultimately leads to RBC destruction in IgG mediated immune hemolysis (warm agglutinins)?

Answer 23

splenic macrophages

Question 24

what process ultimately leads to RBC destruction in IgM mediated immune hemolysis (cold agglutinins)?

Answer 24

complement

Question 25

which plasmodium species has/have daily fevers?

Answer 25

falciparum

Question 26

which plasmodium species has/have every other day fevers?

Answer 26

vivax and ovale