thalassemias

Question 1

microcytic anemia are usually due to

Answer 1

decrease Hb

Question 2

thalassemia is due to

Answer 2

decreased or absent production of globin chains

Question 3

HbA is made of

Answer 3

2 alpha and 2 beta chains

Question 4

HbA2 is made of

Answer 4

2 alpha and 2 delta chains

Question 5

HbF is made of

Answer 5

2 alpha and 2 gamma chains

Question 6

__ genes encode alpha chains

Answer 6

4 genes

Question 7

alpha thalassemia minima

Answer 7

normal red cells
no sxs
carrier

Question 8

alpha thalassemia minor

Answer 8

normal red cells
no sxs
mild anemia

Question 9

alpha minor can be

Answer 9

cis or trans

Question 10

which variant of alpha minor is more likely to affect offspring

Answer 10

cis

Question 11

HbH for

Answer 11

4 beta chains

Question 12

sxs of HbH disease

Answer 12

RBC deformity
extravascular hemolysis

Question 13

HbH disease is dx by

Answer 13

DNA testing

Question 14

treatment for HbH

Answer 14

transfusions

Question 15

Hgb Barts is due to

Answer 15

no alpha chain

Question 16

Hgb barts patients cannot form

Answer 16

HbF

Question 17

Hgb barts consists of

Answer 17

four gamma globin chains

Question 18

sxs of Hgb barts

Answer 18

hydrops fetalis, high output heart failure, and fetal death

Question 19

beta thalassemia is due to

Answer 19

decreased b globin chain synthesis

Question 20

__ genes encode for beta chains

Answer 20

two

Question 21

beta thalassemia is often caused by

Answer 21

mutations

Question 22

beta thalassemia minor will have

Answer 22

increased HgbA2

Question 23

beta thalassemia minor is dx by

Answer 23

electrophoresis

Question 24

beta thalassemia major begins in

Answer 24

first year of life

Question 25

beta thalassemia major sxs

Answer 25

splenomegaly, ineffective erythropoiesis

Question 26

4 characteristics of beta thal major

Answer 26

1. hypo chromic, microcytic anemia
2. abnormal rbc shapes
3. erythroid hyperplasia
4. extrameduallary hematopoiesis

Question 27

what type of rbc are seen in beta thal major

Answer 27

target cells, basophillic stippling

Question 28

target cells are due to

Answer 28

decreased cell vol (thalassemia) or
increased cell membrane (liver dz or splenic dysfunction)

Question 29

sxs of erythroid hyperplasia

Answer 29

chipmunk facies
crew cut appearance
osteoporosis

Question 30

extra medullary hematopoises often produces

Answer 30

nucleated RBCs

Question 31

infection with parvovirus B19 in beta thal may cause

Answer 31

aplastic anemia

Question 32

beta thal major will have what type of Hb presents

Answer 32

HbA2 or HbF

Question 33

alpha and beta thal are protective against

Answer 33

malaria