anemia 2b

Question 1

what happens in alpha globin

Answer 1

any of the 4 alpha genes affected

Question 2

what is MOA of defective gene for alpha thalasemmia

Answer 2

complete deletion

Question 3

what determines the severity of alpha thalasemmia

Answer 3

number of alpha globulin genes lost

Question 4

since alpha is lost, what do the gamma and beta chains do

Answer 4

tetramer result of gamma and beta

Question 5

what happens to a person with loss of all 4 alphas

Answer 5

feta death

Question 6

what is all 4 alpha loss called

Answer 6

hemoglobin Bart

Question 7

what is loss of 3 alpha called

Answer 7

hemoglobin H

Question 8

what happens to the globulins in hemoglobin H

Answer 8

excess beta

tetrameric beta chains

Question 9

for hemoglobin H, what do they show up as in peripheral smear

Answer 9

Heinz bodies

Question 10

what are the shapes of the cell in alpha thalassemia for HbH

Answer 10

teardrop cell

bite cell

Question 11

what is it called when there is a loss of 2 alpha genes

Answer 11

alpha thalassemia traiat

Question 12

what are the 2 types of alpha thalassemia trait

Answer 12

alpha thal 1 - deletion on same gene

alpha thal 2 - deletion of one gene on each chromosome

Question 13

intrinsic hereditary hemolytic anemias: qualitative defect

Answer 13

sickle cell diease

Question 14

what cytoskeleton damage occurs in sickle cell disease

Answer 14

spectrin dimers instead of tetramers

Question 15

hemoglobinopathy

Answer 15

structurally abnormal hemoglobin produced

Question 16

Sickle cell produces what type of hemoglobin

Answer 16

HbS homozygous

Question 17

biochemistry of sickle cell anemia

Answer 17

valine replaces glutamic acid in beta globin chain

Question 18

what are two big clinical presenations for sickle cell

Answer 18

autosplenectomy
chronic leg ulcers
dactylitis
avascular nercrosis 
acute chest syndrome
gallstone formation

Question 19

what bacteria is assoicated with autosplenectomy

Answer 19

salmonella

Question 20

what lab methods are used to determine sickle cell anemia

Answer 20

hemoglobin electrophoresis

HPLC

Question 21

sickle cell interaction with other hemoglobulins HbA

Answer 21

weak interaction, need severe hypoxia to sickle

Question 22

sickle cell interaction with other hemoglobulins HbF

Answer 22

inhibits polymerization, moderate severity

Question 23

what is hemoglobin C

Answer 23

sickling disorder but less severe that sickle cell

Question 24

biochemistry of hemoglobin C

Answer 24

lysine substituted forglutamic acid in beta

Question 25

what body environmental factors affect sickle cell

Answer 25

dehydration increases MCHC

low pH

Question 26

Which thalasemia has HbH inclusions

Answer 26

Alpha thalassemia 1