5.5 - Normocytic anemias with extravascular hemolysis

Question 1

hereditary spherocytosis shows a(n) ______________ (increased / decreased) RDW and a(n) ______________ (increased / decreased) MCHC

Answer 1

• increased

- increased

Question 2

what is seen on blood smear in spherocytosis?

Answer 2

howell-jolly bodies

Question 3

what are howell jolly bodies?

Answer 3

fragments of nuclear material in RBCs

Question 4

what is the inheritance of sickle cell anemia?

Answer 4

AR

Question 5

sickle cell anemia is an AR mutation of the ____ chain of Hb

Answer 5

beta

Question 6

in sickle cell anemia a single amino change replaces normal _________ with ________

Answer 6

• glutamate

- valine

Question 7

how does the replacement of glutamate with valine in sickle cell anemia lead to sickling?

Answer 7

glamatate is hydrophilic whereas valine is hydrophobic

Question 8

sickle cell disease arises when two abnormal ________ genes are present, resulting in over 90% Hb__ in RBCs

Answer 8

• beta

- HbS

Question 9

what is the treatment for sickle cell anemia? why?

Answer 9

hydroxyurea - increases levels of HbF

Question 10

sickle cell patients are at risk of developing what kind of gallstones?

Answer 10

bilirubin gallstones

Question 11

sickle cell patients are at risk of developing osteomyelitis from what type of organism?

Answer 11

salmonella

Question 12

what types of cells are seen on blood smear in sickle cell disease?

Answer 12

• sickle cells

- target cells

Question 13

what screening test is used for both sickle cell disease and trait?

Answer 13

metabisulfite

Question 14

what is the inheritance of HbC?

Answer 14

AR

Question 15

what amino acid is replaced in HbC?

Answer 15

glutamate replaced by lysine