Advanced Hematology Set

1. What is another name for thalassemia major?

A) Cooley's anemia

B) Fanconi's anemia

C) Alpha thalassemia

D) Schilling's syndrome

E) None of the above

A

2. In contrast to beta thalassemia, alpha thalassemia usually manifests:

A) After age 40

B) At the onset of puberty

C) Immediately at birth

D) Only in postmenopausal women

E) After age 10

C

3. Gene deletions are the cause of the majority of alpha thalassemia cases because:

A) There are no areas of homology between the two closely linked alpha genes globin.

B) The a2 globin gene is expressed at two to three times the rate of a1 globin gene.

C) Great homology in the gene blocks render the areas more susceptible to deletion error during crossover.

D) Alpha genes are juxtapositioned to numerous transcriptional mutants.

E) None of the above

C

4. Hemoglobin A consists of:

A) Two alpha (a) chains and two gamma (g) chains

B) Two alpha chains and two delta (d) chains

C) Two alpha chains and two beta (b) chains

D) Two alpha chains and two epsilon (e) chains

E) None of the above

C

5. It has been hypothesized that a person who is heterozygous for the thalassemia gene is resistant to malaria caused by __________.

A) Plasmodium ovale

B) Plasmodium falciparum

C) Plasmodium vivax

D) Plasmodium malariae

E) None of the above

B

6. A person native to Thailand would have more probability of presenting with:

A) Thalassemia major

B) Alpha thalassemia

C) Beta thalassemia

D) Gamma-beta thalassemia

E) None of the above

B

7. What hemoglobin structures make up hemoglobin A2?

A) Zeta 2 epsilon 2

B) Alpha 2 beta 2

C) Alpha 2 gamma 2

D) Alpha 2 delta 2

E) None of the above

D

8. What percentage of normal hemoglobin consists of hemoglobin A?

A) Gene addition

B) Gene fusion

C) Point mutation

D) Point elongation

E) None of the above

C

9. The anemia of thalassemia is morphologically characterized as __________ anemia.

A) Macrocytic/hyperchromic

B) Microcytic/hypochromic

C) Normocytic/normochromic

D) Microcytic/normochromic

E) None of the above

B

10. Which of the following findings would not be indicative of heterozygous b thalassemia?

A) Hemoglobin A2 level of 3.5% to 7%

B) Hemoglobin F level of 2% to 5%

C) Hemoglobin A level of 65% to 85%

D) Hemoglobin A level of 90% to 95%

E) D and A are correct

D

11. Thalassemia is found:

A) Only in the Mediterranean Sea area

B) Only in Australia and South Pacific Islands

C) Only in Southeast Asia

D) Only on the African continent

E) Worldwide

E

12. Which type of bb+ thalassemia produces the least amount of beta chains?

A) Type 1

B) Type 2

C) Type 3

D) Type 4

E) None of the above

A

13. Homozygosity for the b0 or b+ thalassemia gene results in:

A) Hemoglobin H disease

B) Alpha thalassemia

C) Thalassemia major

D) Thalassemia minor

E) None of the above

C

14. The characteristic facial changes in a patient diagnosed with thalassemia major include hypertrophy of maxilla, widely spared eyes, and prominent cheekbones. This is the result of:

A) Expansion of marrow space

B) Extramedullary hematopoiesis

C) Vitamin B12 deficiency

D) Increase in hemoglobins

E) None of the above

A

15. What hemoglobin consists of four gamma chains?

A) Hemoglobin H

B) Hemoglobin Bart's

C) Hemoglobin F

D) Hemoglobin A2

E) None of the above

B

16. Decreased or absent alpha-chain production will result in:

A) Decreased gamma chain production during fetal life and adult life

B) Excess gamma chain production during fetal life and at birth

C) No gamma chain production during fetal life

D) No gamma chain production during adult life

E) None of the above

B

17. Which non-alpha thalassemias may be expected to have similar if not identical hemoglobin A, A2, and F levels?

A) Homozygous hereditary persistence of fetal hemoglobin (HPFH) and homozygous delta-beta thalassemia

B) Homozygous Hgb Lepare and thalassemia intermedia

C) Thalassemia major and thalassemia minor

D) Heterozygous HPFH and homozygous HPFH

E) None of the above

A

18. What lethal disease is characterized by homozygosity of the a0-thalassemia gene haplotype in utero?

A) Hydrops fetalis

B) Hemolytic disease of the newborn (HDN)

C) Hemoglobin Portland disease

D) Hemoglobin H disease

E) Hemoglobin Zurich

A

19. The distinguishing feature of both alpha thalassemia-one-trait and alpha thalassemia-two-trait conditions compared to Bart's hydrops fetalis or hemoglobin H disease is:

A) Lack of hemoglobin Bart's at birth

B) Disappearance of hemoglobin Bart's with development

C) Persistence of high levels of fetal hemoglobin into adulthood.

D) Excessive amounts of hemoglobin Portland at birth

E) None of the above

B

20. All of the following comprise the clinical categories of alpha thalassemia except:

A) Bart's hydrops fetalis syndrome

B) Hemoglobin H disease

C) Hereditary persistence of fetal hemoglobin

D) Alpha thalassemia 1

E) Alpha thalassemia 2

C

21. Which abnormal hemoglobin is produced from a fusion of delta and beta chains?

A) Hemoglobin Lepore

B) Hemoglobin Portland

C) Hemoglobin Constant Spring

D) Hemoglobin Bart's

E) Hemoglobin Zurich

A

22. Which form of HPFH is characterized by uniform distribution among red cells when stained by the Kleinhauer-Betke method?

A) Hypocellular

B) Pancellular

C) Heterocellular

D) Hypercellular

E) Acellular

B

23. What thalassemia-associated variant will result in sickling crisis in severe hypoxic conditions?

A) Beta thalassemia/hemoglobin E

B) Beta thalassemia/hemoglobin C

C) Beta thalassemia/hemoglobin S

D) Alpha thalassemia/sickle cell

E) Hemoglobin SC

C

24. Which clinical expression of thalassemia is associated with a healthy silent carrier showing no clinical symptoms and minimal to no hematologic abnormalities?

A) Thalassemia major

B) Thalassemia minor

C) Thalassemia intermedia

D) Bart's hydrops fetalis

E) None of the above

D

25. Therapy for thalassemia major patients may include:

A) High blood transfusion program

B) Splenectomy

C) Bone marrow transplant

D) All of the above

E) None of the above

D

26. A 5-year-old boy presents with a hemoglobin and hematocrit of 10 g/dL and 30% respectively, a serum bilirubin of 3.0 mg/dL, a RBC count of 4.0 million/µL, and splenomegaly. Which clinical expression of thalassemia best correlates with the data?

A) Thalassemia major

B) Thalassemia minor

C) Thalassemia intermedia

D) Bart's hydrops fetalis

E) HPFH

C

27. The main concern(s) regarding patients with thalassemia major treated with hypertransfusion include(s):

A) Iron overload

B) Alloimmunization

C) Transfusion-transmitted disease

D) All of the above

E) None of the above

D

28. Hemoglobin electrophoresis of a 2-year-old patient showed migration of A2 and F on cellulose acetate at pH 8.4. Quantitation studies showed 5% A2 and 95% F. What thalassemia is most likely present?

A) Alpha thalassemia

B) Beta thalassemia

C) Beta-sickle thalassemia

D) Alpha-sickle thalassemia

E) None of the above

B

29. What is hemoglobin F composed of?

A) Alpha 2, beta 2

B) Alpha 2, gamma 2

C) Alpha 2, delta 2

D) Alpha 2, zeta 2

E) None of the above

B

30. A 13-year-old boy presents with the following hematologic results: RBC = 5.76 million, Hb, 10.4 g/dL; Hct, 35.9%; MCV (mean corpuscular volume), 63 fL: MCH (mean corpuscular hemoglobin), 18.2 pg; MCHC (mean corpuscular hemoglobin concentration), 29%; and RDW (red blood cell distribution width), 13.5. Morphology includes mild anisocytosis and occasional target cells. All iron studies are normal. Electrophoresis on cellulose acetate at pH 8.4 indicates an increase in HbF and HbA2. What thalassemia syndrome is evident?

A) aa-thalassemia

B) bb-thalassemia

C) Hemoglobin E disease

D) Sideroblastic anemia

E) None of the above

B

1. Two-thirds of the total body iron is found as _________.

A) Transferrin

B) Heme in hemoglobin

C) Heme in myoglobin

D) Globin in hemoglobin

E) None of the above

B

2. One-third of the total body iron may be found in storage pools such as:

A) Bone marrow

B) Liver

C) Spleen

D) All of the above

E) None of the above

D

3. What percentage of total body iron is present as plasma/transport iron?

A) 1%

B) 5%

C) 20%

D) 50%

E) 10%

A

4. To carry oxygen iron must be in what state in the heme of the hemoglobin molecule?

A) Ferric (Fe3+)

B) Carboxylated iron

C) Ferrous (Fe2+)

D) Metheme

E) None of the above

C

5. Approximately what percent of ingested iron is absorbed?

A) 1%

B) 5-10%

C) 25-50%

D) 90%

E) None of the above

B

6. Approximately how many milligrams (mg) of iron is lost during pregnancy?

A) 10 mg

B) 100 mg

C) 500 mg

D) 1000 mg

E) 5000 mg

D

7. Which of the following is readily absorbed by intestinal mucosal cells?

A) Ferric iron (Fe3+)

B) Ferrous iron (Fe2+)

C) Hemosiderin

D) All of the above

E) None of the above

B

8. How many milligrams of iron are needed to produce 1 mL of red blood cells (RBCs)?

A) 1 mg

B) 10 mg

C) 20 mg

D) 3 mg

E) 0.5 mg

E

9. For pregnant women, what are the minimum daily requirements for iron and the corresponding iron content of food required?

A) 1.0 mg/10 mg

B) 2.0 mg/20 mg

C) 3.0 mg/30 mg

D) 0.5 mg/5 mg

C

10. For infants, what are the minimal daily requirements for iron and the corresponding iron content of food required?

A) 1.0 mg/10 mg

B) 2.0 mg/20 mg

C) 3.0 mg/30 mg

D) 0.5 mg/5 mg

A

11. What are the minimal daily requirements for iron and the corresponding iron content of food required for men and nonmenstruating females?

A) 1.0 mg/10 mg

B) 2.0 mg/20 mg

C) 3.0 mg/30 mg

D)0.5 mg/5 mg

A

12. What are the minimal daily requirements for iron and the corresponding iron content of food required for menstruating females?

A) 1.0 mg/10 mg

B) 2.0 mg/20 mg

C) 3.0 mg/30 mg

D) 0.5 mg/5 mg

B

13. Iron absorption is influenced by which of the following?

A) Amount and type of iron accessible from food

B) Functional state of the gastrointestinal mucosa and pancreas

C) Current iron stores

D) Erythropoietic needs

E)All of the above

E

14. The majority of the daily iron requirements for production of RBCs is released during daily RBC turnover of aged red cells and is called __________.

A) Recycled iron

B) Stored body iron

C) Myoglobin iron

D) Absorbed iron

E) Hemosiderin

A

15. Which pH facilitates ferrous iron absorption in the small intestines?

A) Alkaline

B) Acidic

C) Neutral

D) Any of the above

E) None of the above

B

16. Which of the following is a beta globulin synthesized in the liver and a transport protein specific for iron?

A) Haptoglobin

B) Albumin

C) Transferrin

D) Hemopexin

E) None of the above

C

17. The storage form of iron is called __________.

A) Ferrous iron

B) Hemopexin

C) Transferrin

D) Ferritin

E) None of the above

D

18. Which of the following is stored in macrophages and can be visualized in bone marrow aspirates by staining with Prussian blue?

A) Hemosiderin

B) Transferrin

C) Hemopexin

D) Haptoglobin

E) None of the above

A

19. Iron-deficiency anemia is characterized by:

A) Macrocytic, hyperchromic RBCs

B) Microcytic, hypochromic RBCs

C) Increased bone marrow storage iron

D) Increased bone marrow ringed sideroblasts

E) None of the above

B

20. Iron-deficiency anemia is characterized by:

A) Decreased serum iron, decreased ferritin, and increased total iron-binding capacity (TIBC)

B) Increased serum iron , increased ferritin, and increased TIBC

C) Decreased serum iron, decreased ferritin, and decreased TIBC

D) Normal serum iron, decreased ferritin, and decreased TIBC

E) None of the above

A

21. Which of the following symptoms may suggest iron-deficiency anemia?

A) Fatigue

B) Lethargy

C) Dizziness

D) All of the above

E) None of the above

D

22. Which of the following laboratory results characterize(s) iron-deficiency anemia?

A) Decreased hemoglobin

B) Decreased MCV (mean corpuscular volume)

C) Decreased MCH (mean corpuscular hemoglobin)

D) Decreased MCHC (mean corpuscular hemoglobin concentration)

E) All of the above

E

23. Which of the following are common causes of iron deficiency anemia worldwide?

A) Chronic blood loss

B) Inadequate diet and malabsorption

C) Pregnancy

D) Excessive menstrual blood loss (premenopausal women)

E) All of the above

E

24. Which of the following characterizes the microcytic, hypochromic anemia of chronic disease or infection?

A) Increases marrow iron

B) Decreases serum iron

C) Normal to increased serum ferritin

D) Decreased TIBC

E) All of the above

E

25. Which of the following disorders represent(s) a problem in forming the RBC hemoglobin molecule, resulting in a microcytic hypochromic anemia?

A) Iron-deficiency anemia

B) Sideroblastic anemia

C) Thalassemia

D) All of the above

E) None of the above

D

26. Which of the following characterize(s) the microcytic hypochromic sideroblastic anemia?

A) Increased ringed sideroblasts

B) Increased serum ferritin

C) Dimorphic blood picture

D) Basophilic stippling

E) All of the above

E

27. Hemosiderosis is the accumulation of excess __________ in the macrophages of various tissues.

A) Iron

B) Heme

C) Hemopexin

D) Globin

E) All of the above

A

28. Disorders commonly associated with anemia of chronic disease include:

A) Infections

B) Malignant neoplasms

C) Autoimmune diseases

D) All of the above

E) None of the above

D

29. Which of the following represent(s) stained iron particles in the RBC?

A) Heinz bodies

B) Howell-Jolly bodies

C) Pappenheimer bodies

D) Cabot rings

E) None of the above

C

30. Which of the following represent(s) an acquired sideroblastic anemia producing a microcytic hypochromic anemia with basophilic stippling, skin lesions, and neurologic dysfunction?

A) Inflammation

B) Lead poisoning

C) Iron-deficiency anemia

D) Anemia of chronic disease

E) All of the above

B

31. Which of the following is (are) characteristic of lead poisoning?

A) Basophilic stippling in RBCs

B) Increased free erythrocyte protoporphyrin (FEP)

C) Microcytic hypochromic anemia

D) All of the above

E) None of the above

D

The normal bone marrow is able to increase its output how many times to compensate for anemia?

6-8 times

The distinguishing feature of both alpha thalassemia-one-trait and alpha thalassemia-two-trait conditions compared to Bart's hydrops fetalis or hemoglobin H disease is:

...

Which of the following should patients be promptly treated with for acquired methemoglobinemia?

Intravenous methylene blue (a strong reducing substance)

In a hemolytic anemia, the first normal compensatory bone marrow response will be reflected in which of the following?

Increase in reticulocyte count

Laboratory features that suggest paroxysmal cold hemoglobinuria include:

All of the above

Hemoglobin A consists of:

Two alpha chains and two beta (β) chains

In HDN, when extramedullary hematopoiesis takes over for the fetal bone marrow to compensate for increased red cell destruction, this results in:

Hepatosplenomegaly

Hemoglobin E occurs with the greatest frequency in which of the following populations?

Asian

What is another name for thalassemia major?

Cooley's anemia

Although sickle cell trait is usually asymptomatic, sickling can still occur when caused by which of the following?

All of the above

Warm autoimmune hemolytic anemia accounts for __________ of all cases of autoimmune hemolytic anemia.

70%

What thalassemia-associated variant will result in sickling crisis in severe hypoxic conditions?

Beta thalassemia/hemoglobin S

Which type of HDN is the most frequent?

ABO HDN

Which of the following abnormal hemoglobins will have a positive tube solubility test?

HgbS

Once a hemolytic anemia is determined, which of the following tests would rule out an immune hemolytic anemia if negative?

Coombs' test (direct antiglobulin test [DAT])

The peripheral blood smear of a patient with warm autoimmune hemolytic anemia will show:

All of the above (I think this is correct)

Which RBC membrane skeletal protein represents a dimer of two highly flexible alpha and beta chains that forms tetramers in the membrane?

Spectrin

Which of the following is characteristic of a hemolytic crisis in a sickle cell anemia patient?

All of the above

In hemoglobin SC disease what is the percentage of HbS and HbC found on the hemoglobin electrophoresis at alkaline pH?

50:50

Most hemoglobin variant traits without coexisting conditions of iron-deficiency anemia or thalassemia have approximately what percentage ratio of normal to abnormal hemoglobin?

60:40

Which of the following is characteristic of HbC disease?

All of the above

Which of the following clinical features suggests a delayed hemolytic transfusion reaction has occurred?

All of the above

The anemias of the hemoglobinopathies such as sickle cell are morphologically classified as:

Normocytic, Normochromic

The major components of the red cell membrane skeleton are:

All of the above

What pathological cold antibody disease occurs in persons older than 50 years of age and demonstrates acrocyanosis of the hands and feet and the presence of a lymphoproliferative disorder?

Cold Agglutinin Syndryome

Which amino acid will differentiate hemoglobin C disease from normal HbA?

Lysine

It has been hypothesized that a person who is heterozygous for the thalassemia gene is resistant to malaria caused by __________.

Plasmodium falciparum

Staining the urine sediment for iron with Prussian blue will detect which of the following during a hemolytic anemia?

Hemosiderin

Hemoglobin C disease is found almost exclusively in which population?

Black

Microangiopathic hemolytic anemia is usually characterized by the presence of:

Schistocytes

Hemolysis induced by drugs is a clinical feature commonly seen in which G6PD deficiency?

Gd Med

Which of the following is the major type of hemolysis characteristic of the majority of cases of HS?

Extravascular hemolysis

Which of the following describes dactylitis, which commonly occurs in infants and young children with sickle cell anemia?

Painful swelling of the hands and feet

The reticulocyte production index (RPI) corrects the hematocrit to a normal value of which percentage, and takes into account the maturation time of the reticulocyte at a particular hematocrit?

45%

Which of the following is characteristic of hemoglobin SC disease?

All of the above

Sickle cell trait represents what inherited form of the disease?

Heterozygous

What hemoglobin structures make up hemoglobin A2?

Alpha 2 delta 2

What hemolytic anemia is a common occurrence in joggers and marathon runners?

March hemoglobinuria

What hemoglobin(s) is/are present in sickle cell trait?

HgS and HgA