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Hematology MCQ’s ( Disorders of Hemoglobin )

Hematology MCQ’s ( Disorders of Hemoglobin ) MCQs Question bank

311 Hemoglobinopathies are disorders that affect which of the following parameters of hemoglobin ?
A. Structure
B. Function
C. Production
D. All of the above
Explanation:- Hemoglobinopathies are disorders affecting the structure, function, or production of hemoglobin

312 The major adult hemoglobin, HbA, has the structure ?
A. alfa 2 beta 2
B. alfa 2 gama 2
C. alfa 2 delta 2
D. None of the above

313 Structure of HbF is ?
A. alfa 2 beta 2
B. alfa 2 gama 2
C. alfa 2 delta 2
D. None of the above

314 Structure of HbA2 is ?
A. alfa 2 beta 2
B. alfa 2 gama 2
C. alfa 2 delta 2
D. None of the above

315 Which of the following about human hemoglobins is false ?
A. alfa -like globin genes are on chromosome 16
B. beta -like globin genes on chromosome 11
C. LCR controlling  globin gene is modulated by ATRX
D. None of the above
Explanation:- Gene clusters encoding human hemoglobins are alpha-like globin genes (on chromosome 16), and beta-like genes (on chromosome 11). Locus control region (LCR) LCR controlling the alphaglobin gene cluster is modulated by ATRX.

316 Which of the following is false ?
A. Normal individual has four  globin genes
B. Normal individual has two  globin genes
C.  and  are -like genes
D. None of the above
Explanation:- A normal individual has 4  globin genes on short arm of chromosome 16 (two genes per chromosome –  / ) & two  globin genes on short arm of chromosome 11 (one per chromosome, or /). The -like genes ( & ) are nearby on chromosome 11.

317 Heme consists of which of the following protoporphyrin ring ?
A. IX
B. X
C. XI
D. XII
Explanation:- Each globin chain has a single heme moiety, consisting of a protoporphyrin IX ring complexed with a single iron atom in ferrous state (Fe2+).

318 Every molecule of hemoglobin can transport how many oxygen molecules ?
A. 1
B. 2
C. 3
D. 4
Explanation:- Each heme moiety can bind a single oxygen molecule. One molecule of hemoglobin can transport up to four oxygen molecules.

319 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
Explanation:- Ballantyne syndrome is also known as mirror syndrome and Triple oedema syndrome.

320 Hemoglobin-oxygen dissociation curve is between percent saturation of Hb and ?
A. pH
B. Tissue PO2
C. Alveolar PO2
D. Tissue PCO2
Explanation:- Hemoglobin-oxygen dissociation curve is between percent saturation of Hb and tissue PO2.

321 Modulator of O2 affinity of heme molecules is ?
A. 2,3-bisphosphoglycerate (2,3-BPG)
B. pH
C. Temperature
D. CO2
Explanation:- Blood pH is the most important modulator of O2 affinity (Bohr effect).

322 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
Explanation:- The Bohr effect is the ability of hemoglobin to deliver more oxygen to tissues at low pH.

323 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
Explanation:- Red cells first appear at about 6 weeks after conception.

324 Which of the following is an embryonic hemoglobin ?
A. Hb Portland
B. Hb Gower I
C. Hb Gower II
D. All of the above
Explanation:- Embryonic hemoglobins are Hb Portland, Hb Gower I and Hb Gower II.

325 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
Explanation:- At 10 – 11 weeks, fetal hemoglobin (HbF) becomes predominant.

326 Almost exclusive synthesis of adult hemoglobin (HbA) in foetus occurs at about ?
A. 28 weeks
B. 30 weeks
C. 34 weeks
D. 38 weeks
Explanation:- Nearly exclusive synthesis of adult hemoglobin (HbA) occurs at ~38 weeks.

327 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
Explanation:- Structural hemoglobinopathies occur when mutations alter the amino acid sequence of a globin chain therby altering physiologic properties of the variant hemoglobins (sickle cell anemia – the most common structural hemoglobinopathy).

328 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
Explanation:- Thalassemia syndromes arise from mutations that impair production or translation of globin mRNA, leading to deficient globin chain biosynthesis.

329 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

330 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

331 In sickle cell syndrome, HbS is best illustrated as ?
A. alfa 2 beta 2
3 Glu -> Va1
B. alfa 2 beta 2
4 Glu -> Va1
C. alfa 2 beta 2
5 Glu -> Va1
D. alfa 2 beta 2
6 Glu -> Va1
Explanation:- The sickle cell syndromes are caused by a mutation in the beta-globin gene that changes the sixth amino acid from glutamic acid to valine. Whereas thalassemias are caused by gene mutations resulting in decreased production of  or  globin chains, the sickle cell disorders are hemoglobinopathies caused by mutations in the  globin gene resulting in the production of a structurally abnormal  globin chain.

332 HbC is best illustrated as ?
A. alfa 2 beta 2
3 Glu -> Lys
B. alfa 2 beta 2
4 Glu -> Lys
C. alfa 2 beta 2
5 Glu -> Lys
D. alfa 2 beta 2
6 Glu -> Lys

333 Hand-foot syndrome is related to which of the following ?
A. Tietze Syndrome
B. Hypertrophic Osteoarthropathy
C. Sickle cell disease
D. Syringomyelia
Explanation:- Sickle cell dactylitis or hand-foot syndrome caused by painful infarcts of digits & dactylitis is seen in sickle cell disease & sickle cell thalassemia below the age 4 or 5 years & not in adults.

334 Bone pain in sickle cell crisis is due to ?
A. Fracture
B. Bone & bone marrow infarction
C. Hyperuricemia
D. Osteoporosis
Explanation:- The bone pain in sickle cell crisis is due to bone and bone marrow infarction.

335 Which of the following is useful in the treatment of sickle cell disease ?
A. Anagrelide
B. Danazol
C. IFN-alpha
D. Hydroxyurea
Explanation:- Hydroxyurea (10 – 30 mg/kg per day) increases fetal hemoglobin, has favourable effects on RBC hydration, vascular wall adherence, and suppresses granulocyte & reticulocyte counts.

336 Which of the following drugs may elevate HbF ?
A. Hydroxyurea
B. 5-azacytidine
C. 5-deoxyazacytidine (decitabine)
D. All of the above
Explanation:- Antitumor drug 5-azacytidine was the first agent found to elevate HbF. Its widespread use has been prevented because of concerns about acute toxicity & carcinogenesis. Low doses of 5- deoxyazacytidine (decitabine) can elevate HbF with more acceptable toxicity.

337 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
Explanation:- Muddy appearance of freshly drawn blood is characteristic of Methemoglobinemia.

338 Which of the following facies is typical of thalassemia ?
A. Plethoric moon facies
B. Leonine facies
C. Chipmunk facies
D. Elfin facies
Explanation:- In thalassemic children, characteristic “chipmunk” facies occurs due to maxillary marrow hyperplasia & frontal bossing.

339 In -thalassemia-2 trait, how many of the four -globin loci are deleted ?
A. 1
B. 2
C. 3
D. 4

340 In -thalassemia-1 trait, how many of the four -globin loci are deleted ?
A. 1
B. 2
C. 3
D. 4

341 In hydrops fetalis with Hb Bart’s, how many of the four -globin loci are deleted ?
A. 1
B. 2
C. 3
D. 4

342 In HbH disease, how many of the four -globin loci are deleted ?
A. 1
B. 2
C. 3
D. 4
Explanation:- The four classic  thalassemias are -thalassemia-2 trait (one of the four -globin loci is deleted), -thalassemia-1 trait (with two deleted loci), HbH disease (with three loci deleted) and hydrops fetalis with Hb Bart’s (all four loci deleted).

343 Which of the following -thalassemia resembles -thalassemia minor ?
A. -thalassemia-1 trait
B. -thalassemia-2 trait
C. HbH disease
D. Hydrops fetalis with Hb Bart’s
Explanation:- -thalassemia-1 trait resembles -thalassemia minor. -thalassemia-2 trait is an asymptomatic, silent carrier state.

344 Hb Barts is designated as ?
A. 4
B. 4
C. 4
D. 4
Explanation:- Hb Barts is designated as 4

345 Which of the following is not a feature of thalassemia ?
A. Avascular necrosis
B. Osteomalacia
C. Osteopenia
D. Microfractures
Explanation:- Avascular necrosis is not a feature of thalassemia because there is no sickling of red cells leading to thrombosis and infarction.

346 In sickle cell disease, presence of palpable spleen after what age suggests a coexisting thalassemia ?
A. 5 years
B. 10 years
C. 15 years
D. 20 years
Explanation:- Presence of a palpable spleen in sickle cell disease after age 5 years suggests a coexisting hemoglobinopathy like thalassemia.

347 Which of the following is a cause of microcytic erythrocytosis ?
A. Thalassemia trait
B. Hypoxic erythrocytosis
C. Polycythemia vera (PV)
D. All of the above
Explanation:- Only 3 conditions cause microcytic erythrocytosis – thalassemia trait, hypoxic erythrocytosis & PV. RDW is normal in beta-thalassemia, while it is elevated in hypoxic erythrocytosis & PV.

348 Target cells in PBF can be seen in which of the following conditions ?
A. Thalassemia
B. Iron deficiency
C. Cholestatic liver disease
D. All of the above
Explanation:- Target cells (area of central pallor with dense center or bull’s eye) is typical of thalassemia, but can be seen in iron deficiency, cholestatic liver disease or as an artifact. Larger numbers are typical of hemoglobin C disease.

349 Geographic distributions of which of the following closely resemble that of malaria ?
A. Sickle cell disease
B. Thalassemia
C. Glucose-6-phosphate dehydrogenase (G6PD) deficiency
D. All of the above
Explanation:- Geographic distributions of sickle cell disease, ovalocytosis, thalassemia, and glucose-6-phosphate dehydrogenase (G6PD) deficiency closely resemble that of malaria

350 Which of the following is normal or increased in thalassemia ?
A. Serum iron level
B. Transferrin saturation
C. Serum ferritin level
D. All of the above
Explanation:- Normal or increased serum iron & ferritin levels and transferrin saturation are characteristic of the thalassemias.

351 Cooley’s anemia refers to ?
A. Beta thalassemia minor
B. Beta thalassemia intermedia
C. Beta thalassemia major
D. Any of the above
Explanation:- In -thalassemia, the  globin chains are structurally normal but quantitatively reduced.

352 Variant hemoglobins that may be co-inherited with - thalassemia are ?
A. Hemoglobin S
B. Hemoglobin E
C. Hemoglobin C
D. All of the above
Explanation:- Variant hemoglobins that may be co-inherited with -thalassemia are Hemoglobin S, Hemoglobin E and Hemoglobin C.

353 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
Explanation:- Hemoglobin E is referred to as a “thalassemic hemoglobinopathy” because in addition to being structurally abnormal it is produced in reduced quantities. Patient who inherits a HbE mutation from one parent and a -thalassemia mutation from another (HbE/-thalassemia) will clinically be similar to a patient with -thalassemia intermedia or major.

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

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

356 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”
Explanation:- Hepcidin is a small peptide that inhibits iron absorption in small bowel. Hepcidin levels normally increase when iron stores are elevated. Hepcidin levels are inappropriately low in thalassemia intermedia & thalassemia major.

357 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
Explanation:- The finding of a normal MCV (>= 80 fL) with normal MCH (>= 27 pg) and normal Hb electrophoresis or HPLC rules out most cases of thalassemia and requires no additional thalassemia testing.

358 Patients with -thalassemia trait have an elevated HbA2 of ?
A. > 0.5 %
B. > 1.5 %
C. > 2.5 %
D. > 3.5 %
Explanation:- Patients with -thalassemia trait have an elevated HbA2 of > 3.5 %.

359 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

360 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
Explanation:- A unit (250-300 ml) of packed RBCs contains 250 – 300 mg of iron (1 mg/mL).

361 Patients develop hemosiderosis after how many units of packed RBCs ?
A. > 50 units
B. > 100 units
C. > 200 units
D. > 500 units
Explanation:- Patients who receive >100 units of packed RBCs usually develop hemosiderosis.

362 Which of the following is an not an oral iron-chelating agent ?
A. Deferoxamine
B. Deferiprone
C. Deferasirox
D. None of the above
Explanation:- Deferoxamine is iron-chelating agent & requires parenteral administration.

363 Which of the following promotes high levels of HbF synthesis ?
A. Hydroxyurea
B. Butyrates
C. Cytarabine
D. All of the above
Explanation:- Reestablishing high levels of HbF synthesis can ameliorate symptoms of thalassemia. Hydroxyurea & cytarabine promote high levels of HbF synthesis by stimulating proliferation of F cell progenitors. Butyrates also stimulate HbF production.

364 Lucarelli classification is used to classify ?
A. Porphyria
B. Hemolytic anemia
C. Thalassemia
D. Anemia
Explanation:- Lucarelli classification assess risk factors that predict outcome & prognosis in thalassemia.

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
Explanation:- Vitamin C should not be supplemented in iron excess states because it generates free radicals.

366 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
Explanation:- Precipitates of unpaired  chains form single large inclusions known as Heinz bodies.

367 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
Explanation:- Cardiothoracic ratio in fetal ultrasound is used to assess risk of hemoglobin Bart’s hydrops fetalis (normal < 0.53).

368 Estimated 5-year survival rate following allogeneic bone marrow transplantation is ?
A. 30 %
B. 50 %
C. 70 %
D. 90 %
Explanation:- Estimated 5-year survival rate following allogeneic bone marrow transplantation is 90% if done before they develop hepatomegaly or portal fibrosis and if given adequate iron chelation therapy.

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