[MCQs] Disorders of Hemoglobin – Part 2

The quizzes about Renal Function (32 questions)[Immunology] The Immunoproliferative Diseases Quizzes (19 tests)I. Start the exam by click the “Start” button

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

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1. In a-thalassemia-1 trait, how many of the four a-globin loci are deleted ?

1. 1
2. 2
3. 3
4. 4

2. In a-thalassemia-1 trait, how many of the four a-globin loci are deleted ?

1. 1
2. 2
3. 3
4. 4

3. In hydrops fetalis with Hb Bart’s, how many of the four a-globin loci are deleted ?

1. 1
2. 2
3. 3
4. 4

4. In HbH disease, how many of the four a-globin loci are deleted ?

1. 1
2. 2
3. 3
4. 4

5. Which of the following a-thalassemia resembles P-thalassemia minor ?

1.  a-thalassemia-1 trait
2. a-thalassemia-2 trait
3. HbH disease
4. Hydrops fetalis with Hb Bart’s

6. Hb Barts is designated as ?

1. a₄
2. P4
3. Ĩ4
4. §4

7. Which of the following is not a feature of thalassemia ?

1. Avascular necrosis
2. Osteomalacia
3. Osteopenia
4. Microfractures

8. In sickle cell disease, presence of palpable spleen after what age suggests a coexisting thalassemia ?

1.  5 years
2. 10 years
3. 15 years
4. 20 years

9. Which of the following is a cause of microcytic erythrocytosis ?

1. Thalassemia trait
2. Hypoxic erythrocytosis
3. Polycythemia vera (PV)
4. All of the above

10. Target cells in PBF can be seen in which of the following conditions ?

1. Thalassemia
2. Iron deficiency
3. Cholestatic liver disease
4. All of the above

11. Geographic distributions of which of the following closely resemble that of malaria ?

1. Sickle cell disease
2. Thalassemia
3. Glucose-6-phosphate dehydrogenase (G6PD) deficiency
4. All of the above

12. Which of the following is normal or increased in thalassemia ?

1. Serum iron level
2. Transferrin saturation
3. Serum ferritin level
4. All of the above

13. Cooley’s anemia refers to ?
A. Beta thalassemia minor
B. Beta thalassemia intermedia
C. Beta thalassemia major
D. Any of the above

14.  Variant hemoglobins that may be co-inherited with β– thalassemia are ?
A. Hemoglobin S
B. Hemoglobin E
C. Hemoglobin C
D. All of the above

15.  Which of the following is referred to as a “thalassemic hemoglobinopathy” ?
A. Hemoglobin S
B. Hemoglobin E
C. Hemoglobin C
D. All of the above

16.  Features of thalassemia include all except ?
A. Hypopituitarism
B. Hypogonadism
C. Hypoparathyroidism
D. Diabetes insipidus

17.  Features of thalassemia include all except ?
A. Cardiomyopathy
B. Excessive melanin skin pigmentation
C. Pulmonary fibrosis
D. Diabetes mellitus

18.  Which of the following about Hepcidin is false ?
A. Inhibits iron absorption in small bowel
B. Levels increase when iron stores are elevated
C. Levels are high in patients with thalassemia major
D. Also called “Storage iron regulator”
.
19.  Which of the following findings rules out thalassemia and no additional thalassemia testing is required ?
A. MCV > 80 fL, MCH > 27 pg, normal Hb electrophoresis
B. MCV > 90 fL, MCH > 27 pg, normal Hb electrophoresis
C. MCV > 100 fL, MCH > 27 pg, normal Hb electrophoresis

D. MCV > 110 fL, MCH > 27 pg, normal Hb electrophoresis

20. Patients with β-thalassemia trait have an elevated HbA2 of ?
A. > 0.5 %
B. > 1.5 %
C. > 2.5 %
D. > 3.5 %

21.  Which of the following is not applicable for estimating iron overload in thalassemia patients ?
A. Serum ferritin
B. Magnetic susceptometry (SQUID)
C. Labile plasma iron estimation
D. CT of liver

22.  A unit (250-300 ml) of packed RBCs contains how much iron ?
A. 50 – 100 mg
B. 100 – 150 mg
C. 150 – 250 mg
D. 250 – 300 mg

23.  Patients develop hemosiderosis after how many units of packed RBCs ?
A. > 50 units
B. > 100 units
C. > 200 units
D. > 500 units

24.  Which of the following is an not an oral iron-chelating agent ?
A. Deferoxamine
B. Deferiprone
C. Deferasirox
D. None of the above

25.  Which of the following promotes high levels of HbF synthesis ?
A. Hydroxyurea
B. Butyrates
C. Cytarabine
D. All of the above

26. Lucarelli classification is used to classify ?
A. Porphyria
B. Hemolytic anemia
C. Thalassemia
D. Anemia

27. 365 Which of the following should be avoided in an iron excess state ?
A. Vitamin C
B. Vitamin E
C. Folic acid
D. Plant flavonoids

28.  Heinz bodies is best related to ?
A. Precipitates of unpaired  globin chains
B. Precipitates of unpaired a globin chains
C. Precipitates of unpaired a & b globin chains
D. Any of the above

29.  Which parameter in fetal ultrasound is used to assess risk of hemoglobin Bart’s hydrops fetalis ?
A. Abdominal circumference
B. Nuchal translucency
C. Cardiothoracic ratio
D. Ventricular system of fetal brain

30.  Estimated 5-year survival rate following allogeneic bone marrow transplantation is ?
A. 30 %
B. 50 %
C. 70 %
D. 90 %