[MCQs] Disorders of Hemoglobin – Part 1

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Disorders of Hemoglobin – Part 1 | Disorders of Hemoglobin – Part 2

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1. Hemoglobinopathies are disorders that affect which of the following parameters of hemoglobin ?
A. Structure
B. Function
C. Production
D. All of the above

2. The major adult hemoglobin, HbA, has the structure ?

1.   a₂P₂
2. aY
3. a₂S₂
4. None of the above

3. Structure of HbF is ?

1. a₂P₂
2. aY
3. a₂S₂
4. None of the above

4.  Structure of HbA2 is ?

1. a₂P₂
2. aY₂
3. a₂S₂
4. None of the above

5.  Which of the following about human hemoglobins is false ?

1. a-like globin genes are on chromosome 16
2. P-like globin genes on chromosome 11
3. LCR controlling a globin gene is modulated by ATRX
4. None of the above

6.  Which of the following is false ?

1. Normal individual has four a globin genes
2. Normal individual has two P globin genes
3. Y and S are p-like genes
4. None of the above

7.  Heme consists of which of the following protoporphyrin ring ?
A. IX
B. X
C. XI
D. XII

8.  Every molecule of hemoglobin can transport how many oxygen molecules ?

A. 1
B. 2
C. 3
D. 4

9. Severe fetal hydrops is related to which of the following ?
A. Ballantyne syndrome
B. Mirror syndrome
C. Triple oedema syndrome
D. All of the above

10. Hemoglobin-oxygen dissociation curve is between percent aturation of Hb and ?
Harrison’s 18th Ed. 853 Figure 104-2

1. pH
2. Tissue PO₂
3. Alveolar PO₂
4. Tissue PCO₂O2

11.  Modulator of O2 affinity of heme molecules is ?
A. 2,3-bisphosphoglycerate (2,3-BPG)
B. pH
C. Temperature
D. CO2

12.  Bohr effect is the ability of hemoglobin to deliver more oxygen to tissues at ?
A. Low pH
B. Neutral pH
C. High pH
D. Any of the above

13.  In which week of gestation red cells first appear in foetus ?
A. About 2 weeks
B. About 4 weeks
C. About 6 weeks
D. About 8 weeks

14 Which of the following is an embryonic hemoglobin ?
A. Hb Portland
B. Hb Gower I
C. Hb Gower II
D. All of the above

15 In which week of gestation does fetal hemoglobin become predominant ?
A. About 6 weeks
B. About 10 weeks
C. About 18 weeks
D. About 24 weeks

16.  Almost exclusive synthesis of adult hemoglobin (HbA) in foetus occurs at about ?

A. 28 weeks
B. 30 weeks
C. 34 weeks
D. 38 weeks

17. When mutations alter the amino acid sequence of a globin chain, which of the following hemoglobinopathies occur ?
A. Structural hemoglobinopathies
B. Thalassemia syndromes
C. Hereditary persistence of fetal hemoglobin (HPFH)
D. Acquired hemoglobinopathies

18. When mutations impair production or translation of globin mRNA, which of the following hemoglobinopathies occur ?
A. Structural hemoglobinopathies
B. Thalassemia syndromes
C. Hereditary persistence of fetal hemoglobin (HPFH)
D. Acquired hemoglobinopathies

19.  Which of the following statements about hemoglobinopathies is false ?
A. Common in malaria endemic areas
B. Thalassemia children more susceptible to infection with
nonlethal Plasmodium vivax
C. Thalassemia naturally protects against infection with
Plasmodium falciparum
D. None of the above

20. Which of the following is not a characteristic of Sickle Cell Syndromes ?
A. Microvascular vasoocclusion
B. Premature RBC destruction
C. Stiff RBC membrane
D. None of the above

21. HbC is best illustrated as ?
Harrison’s 18th Ed. 854

1. a₂P₂³
2. a₂P₂⁴
3. a₂P₂⁵
4. a₂P₂⁶

22.  Hand-foot syndrome is related to which of the following ?
A. Tietze Syndrome
B. Hypertrophic Osteoarthropathy
C. Sickle cell disease
D. Syringomyelia

23. Bone pain in sickle cell crisis is due to ?
A. Fracture
B. Bone & bone marrow infarction
C. Hyperuricemia
D. Osteoporosis

24.  Which of the following is useful in the treatment of sickle cell disease ?
A. Anagrelide
B. Danazol
C. IFN-alpha
D. Hydroxyurea

25.  Which of the following drugs may elevate HbF ?
A. Hydroxyurea
B. 5-azacytidine
C. 5-deoxyazacytidine (decitabine)
D. All of the above

26.  Muddy appearance of freshly drawn blood is characteristic of which of the following ?
A. Sickle cell anemia
B. Methemoglobinemia
C. Thalassemia
D. All of the above

27.  Which of the following facies is typical of thalassemia ?

A. Plethoric moon facies

B. Leonine facies
C. Chipmunk facies
D. Elfin facies

28.In a-thalassemia-2 trait, how many of the four a-globin loci are deleted ?

A. 1
B. 2
C. 3
D. 4