Chapter 5-6

Question 1

Hereditary spherocytosis what type of anemia?

Answer 1

normocytic anemia w/ predominant extravascular hemolysis

Question 2

Hereditary spherocytosis mutation?

Answer 2

mutation in spectrin, ankyrin, band 3.1

Question 3

smear lab findings of Hereditary spherocytosis

Answer 3

round loss of central pallor & target cells.
increase RDW
increase MCHC

Question 4

clinical findings of Hereditary spherocytosisa

Answer 4

splenomegally, jaundice, unconjugate bilirubin increases risk of bilirubin gallstone.

Question 5

pts with HS are increased risk of what?

Answer 5

Aplastic crisis with parvovirus B19 infection of erythroid precursor.

Question 6

diagnose Hereditary spherocytosis

Answer 6

osmotic fragility test increased spherocyte fragility in hypotonic soln=burst normally wouldnt burst

Question 7

tx of hereditary spherocytosis

Answer 7

splenectomy but appearance of howell jolly bodies normally removed by spleen

Question 8

Sickle cell anemia

Answer 8

AR mutation of BETA chain of hemoglobin

Glu/hydrophilic–>6 Val/hydrophobic

Question 9

carriers of sickle anemia are resistant?

Answer 9

falciparum maleria

Question 10

HBs results in alpha 2 beta s on both

Answer 10

HbS polymerizes when deoxygenated into needle like structure

Question 11

sickling increases in what setting

Answer 11

hypoxemia dehydration and acidosis

Question 12

what is used in tx of sickle cell

Answer 12

Hydroxyurea increases HbF protects against sickling

Question 13

result of sickle cell leading to extravascular hemolysis

Answer 13

reticuloendothelial system removes RBCs leads to anemia jaundice, unconjugated biliriubin and bilirubin gallstones

Question 14

sickle cell lead to massive erythroid hyperplasia s/s

Answer 14

crewcut on x-ray and facial bones chipmunk facies- expansion of hematopoiesis, and hepatomegaly & risk of aplasitic crisis w parvoB19

Question 15

hallmark for infant with sickle cell anemia

Answer 15

black baby with dactylitis - swollen hands and feet
or
autosplenectomy

Question 16

most common cause of death in adult pts with sickle cell

Answer 16

acute chest syndrome- chest pain SOB, lung infiltrats, precipitated by pneumonia

Question 17

sickle cell trait

Answer 17

usually no sickling unless under stress, only one of the Beta chains in sickle.

Question 18

screen for sickle cell

Answer 18

metabisulfite causes cells with any HbS to sickle positive in both disease and trait.

Question 19

hemoglobin C

Answer 19

Glu 6 LYSINE=crystals mild anemia due toe extravascular hemolysis