Hema Lec #11: Thalassemima

Question 1

T/F. Normal hemoglobin structure are made up of 4 globular protein subunits, each with one molecule of heme and each heme with one iron ion

Answer 1

true

Question 2

Which of the following are not referred to the statement?

a. two alpha chains in hemoglobin A are encoded by an identical pair of alpha globin genes on chromosome 11
b. two beta chains are encoded by a single beta-globin gene on chromosome 16
c. none of the above
d. two beta chains are encoded by a identical pair of beta-globin gene on chromosome 16
e. two alpha chains in hemoglobin A are encoded by a single alpha globin genes on chromosome 11

Answer 2

C. none of the above

Question 3

What are the chain composition of the form HbA

Answer 3

alpha2beta2

Question 4

What are the chain composition of the form HbA2?

Answer 4

alpha2delta2

Question 5

What are the chain composition of the form HbF?

Answer 5

alpha2gamma2

Question 6

What variant of hemoglobin are abundant in total hmeoglobin

Answer 6

HbA

Question 7

T/F. Thalassemia are homogenous group of disorders caused by inherited murations that decrease the synthesis of either alpha-globin chain or beta globin chain that composes adult hemogblon

Answer 7

false. heterogenous

Question 8

Thalassemia leads to …

Answer 8

anemia, tissue hypoxia and red cell hemolysis

Question 9

T/F. Thalassemia is epidemic in Mediterranean basin, Middle East, tropical Africa, Indian subcontinent and Asia

Answer 9

False. endemic

Question 10

What is the reason when one chain increased levels as the other chain decreased its level

a. to compensate for the reduced chain
b. due to relative excess of the other globin chain
c. due to Beta-thalassemia
d. all of the above
e. only A is correct

Answer 10

d. all of the above

Question 11

clasify the anemia as major, intermedia and minor accordign to their clinical characteristics

a. transfusion independent
b. transfusion dependent
c. no clinical symptoms but with hematologic abnormalities

Answer 11

a. Intermedia
b. major
c. minor

Question 12

What type of thalassemia that is most commonly known by point mutation on chromosome 11 and has less common;y deletion of part of the gene

Answer 12

Beta thalassemia

Question 13

A mutation with absent Beta globin synthesis

Answer 13

beta null mutation

Question 14

A mutation with less or reduced but detectable BEta globin synthesis

Answer 14

Bets plus mutation

Question 15

What is the proximal cause of most red cell pathology

a. Imbalance between alpha and beta globin chain which deficit in HbA synthesis produces hypochromic, microcytic red cells
b. Decrease beta globin synthesis lead to unpaired alpha chains precipitate with insoluble inclusions
c. due to apoptosis

Answer 15

b. Decrease beta globin synthesis lead to unpaired alpha chains precipitate with insoluble inclusions

Question 16

T/F. RBCs that contain inclusions and have membrane damage are prone to splenic sequestration and extravascular hemolysis in the spleen

Answer 16

True

Question 17

Which of the following does not refer to the complications in Beta thalassemia d/t ineffective erythropoiesis

a. produces skeletal abnormalities
b. extensive extramedullary hematopoiesis that involves in spleen, liver, trachea and lymph nodes
c. cause cachexia in untreated patients
d. supresses hepcidin

Answer 17

b. extensive extramedullary hematopoiesis that involves in spleen, liver, trachea and lymph nodes

except sa trachea